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Journal articles on the topic 'Hypoplastic heart syndromes'

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Author: Grafiati

Published: 11 March 2023

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1

REIS, PATRICIA M., MARGARET R. PUNCH, EDWARD L. BOVE, and COSMAS J. M. VAN DE VEN. "Outcome of Infants With Hypoplastic Left Heart and Turner Syndromes." Obstetrics & Gynecology 93, no. 4 (April 1999): 532–35. http://dx.doi.org/10.1097/00006250-199904000-00012.

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2

Reis, P. "Outcome of infants with hypoplastic left heart and turner syndromes." Obstetrics & Gynecology 93, no. 4 (April 1999): 532–35. http://dx.doi.org/10.1016/s0029-7844(98)00462-1.

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3

Caliebe, A., J. Hansen, K. Becker, U. M. M. Bauer, H. H. Kramer, and M. P. Hitz. "Types of Recognisable Syndromes Observed in Patients with Hypoplastic Left Heart Syndrome (HLHS)." Thoracic and Cardiovascular Surgeon 65, S 02 (February 2, 2017): S111—S142. http://dx.doi.org/10.1055/s-0037-1599031.

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4

Turturro, Francesco, Cosma Calderaro, Antonello Montanaro, Luca Labianca, Giuseppe Argento, and Andrea Ferretti. "Isolated Asymptomatic Short Sternum in a Healthy Young Girl." Case Reports in Radiology 2014 (2014): 1–3. http://dx.doi.org/10.1155/2014/761582.

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Congenital sternal defects are rare deformities frequently associated with other anomalies of the chest wall and other organ systems. Although pectus excavatum, pectus carinatum, and cleft sternum can present as isolated deformity, in most cases they are associated with heart and inner organs anomalies and described as symptoms of syndromes like Marfan syndrome, Noonan syndrome, Poland anomaly, and Cantrell pentalogy. In contrast, the etiology of an isolated defect is not well understood. We observed a short sternum (dysmorphic manubrium, hypoplastic body, and complete absence of the xiphoid process) in a completely asymptomatic 13-year-old woman. A comprehensive instrumental exams panel was performed to exclude associated anomalies of the heart and of the other organ systems. The patient was completely asymptomatic and she did not need any medical or surgical treatment. To our knowledge, this is the first case of isolated short sternum reported in literature.

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5

Mohamed Ahmed, Eltayeb, Alexandru Ciprian Visan, Graham Stuart, and Serban Stoica. "Aortic root and hemiarch replacement in a patient with Loeys–Dietz and hypoplastic left heart syndromes†." Interactive CardioVascular and Thoracic Surgery 26, no. 2 (October 11, 2017): 346–47. http://dx.doi.org/10.1093/icvts/ivx336.

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6

Annigeri, Rajeshwari G., V. V. Subba Reddy, G. P. Mamatha, Manisha Jadhav, and P. Poornima. "Ellis-van Creveld syndrome affecting siblings: A case report and review." CODS Journal of Dentistry 6, no. 1 (2014): 40–44. http://dx.doi.org/10.5005/cods-6-1-40.

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Abstract Ellis-van Creveld syndrome (EVC) is a genetic disorder that was first described by Richard Ellis and Simon van Creveld in 1940. EVC is a rare autosomal recessive disease resulting from a genetic defect located in chromosome 4p16. The name chondroectodermal is used as it affects both the skeleton (chondro) and the skin (ectoderm). The four principal characteristics are chondrodysplasia, polydactyly, ectodermal dysplasia and congenital heart defects. The patients have small stature, short limbs, fine sparse hair and hypoplastic nails. The orofacial manifestations include multiple gingivolabial musculofibrous frenule, dental anomalies like hypodontia associated with malocclusion. This entity can be diagnosed at any age, even during pregnancy. The differentiation should be made between Asphyxiating Thoracic Dysplasia (Jeune syndrome) and other orofaciodigital syndromes. A multidisciplinary approach is required to manage this condition. We are reporting a rare clinical entity of chondroectodermal dysplasia with classical signs affecting siblings who reported to the Department of Oral Medicine and Radiology with review of its literature. How to cite this article Mamatha GP, Manisha J, Rajeshwari GA, Poornima P, Subba Reddy VV. Ellis-van Creveld syndrome affecting siblings – A case report and review. CODS J Dent 2014;6;40-44

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7

Anderson, Robert H., Audrey Smith, and Andrew C. Cook. "Hypoplasia of the left heart." Cardiology in the Young 14, S1 (February 2004): 13–21. http://dx.doi.org/10.1017/s1047951104006249.

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The lesion that, nowadays, is most usually described as “hypoplastic left heart syndrome”, was initially described in terms of “hypoplasia of the aortic outflow tract complex”.1 Sporadic cases with aortic atresia, an intact ventricular septum, and gross hypoplasia of the left ventricle, had been described long before, but it was Noonan and Nadas, in a landmark study, who coined the term “hypoplastic left heart syndrome”.2 The paediatric cardiac community has now accepted this term uniformly, although as we will see, problems remain with regard to precisely which malformations should be included within the “syndrome”. The term itself, nonetheless, is not beyond criticism. This is because, for those working in the genetic community, a “syndrome”, by definition, is a constellation of anomalies afflicting multiple systems of organs. The so-called “hypoplastic left heart syndrome”, however, almost always involves only the heart and the great arteries. Thus, according to the geneticists, it should not strictly be described as a “syndrome”, although the Nomenclature committee of the International Coding Project have marshalled arguments in favour of the term. In this review, nonetheless, we will skirt these problems with the use of “syndrome”, and simply describe the morphology as seen in patients unified because they have hypoplasia of the left heart.

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8

Jessie, Evangelista E., Mary M. Ruisi, Daniel Green, Rachel Burt, Jaclyn Davis, Regina A. Macatangay, Farid Boulad, et al. "Radioulnar Synostosis-Hematology (RUS-H) Syndrome: Description of the New Syndrome and Comparison to Similar Syndromes." Blood 120, no. 21 (November 16, 2012): 1100. http://dx.doi.org/10.1182/blood.v120.21.1100.1100.

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Abstract Abstract 1100 Congenital radioulnar synostostis (RUS) is a rare anomaly characterized by fusion of the radius and ulna. RUS occurs more frequently in males than females, and is bilateral in 50% of cases. Since 1793, there have been > 400 cases reported. Literature review revealed 7 rare syndromes with RUS and hematologic problems including our newly named RUS-H Syndrome: 1) Diamond-Blackfan Anemia (DBA) associated with normochromic, macrocytic anemia in early infancy and erythroblastopenia; 2) Amegakaryocytic Thrombocytopenia Radioulnar Syndrome (ATRUS, HoxA11 mutation) with thrombocytopenia since birth requiring stem-cell transplantation; 3) IVIC Syndrome with mild thrombocytopenia and leukocytosis; 4) WT Syndrome involving a wide array of hematologic abnormalities including easy bruising, hypoplastic anemia, pancytopenia, Acute Lymphoblastic Leukemia (ALL), and Acute Myeloblastic Leukemia (AML); 5) Cohen Syndrome with neutropenia and fluctuating thrombocytopenia; 6) Noonan Syndrome with abnormal bleeding and easy bruising; and 7) RUS-H Syndrome (not involving HoxA11 mutations) associated with a spectrum of hematologic abnormalities including easy bruising, recurrent epistaxis, neutropenia, thrombocytopenia, ALL and aplastic anemia. This is a subsequent report to our ASH 2010 abstract. All 7 syndromes are associated with hand abnormalities. Six of the 7 syndromes (not Cohen) are associated with hearing loss/ear abnormalities. Four syndromes (DBA, ATRUS, WT, and RUS-H) have an increased risk of hematological malignancy. DBA, IVIC, Cohen, and Noonan have abnormalities of the eye and genitourinary system. DBA, WT, Cohen, and Noonan Syndromes are associated with dysmorphic facial features. ATRUS, IVIC, Cohen, and Noonan Syndromes all exhibit lower limb abnormalities. DBA, Cohen, Noonan, and RUS-H are associated with short stature. DBA, IVIC, Noonan, and RUS-H Syndromes are associated with kidney abnormalities and structural heart defects. Three Syndromes (DBA, IVIC, and Cohen) have cranial abnormalities. DBA IVIC, and Noonan Syndrome have structural defects of the shoulder. IVIC, Cohen, and Noonan Syndromes are associated with spinal anomalies. Two Syndromes (DBA and Noonan Syndrome) are associated with liver, spleen, and neck abnormalities. Cohen and Noonan Syndrome are associated with developmental delays. Lastly, WT and Noonan Syndrome are associated with skin abnormalities (Figure 1). This abstract highlights the association of RUS, other congenital abnormalities, and hematologic problems in previously described syndromes and in the novel RUS-H Syndrome. DBA, Cohen, and Noonan Syndrome are the most common of the 7 syndromes, with DBA estimated at 5 per 1,000,000; Cohen Syndrome predicted to have a prevalence of <1,000; and Noonan Syndrome predicted to have a prevalence of <1 in 2,500; however, RUS in DBA, Cohen, and Noonan Syndrome is limited to case reports. RUS-H Syndrome has been identified in 12 families in the United States, Canada, and England. IVIC and WT Syndromes have been reported in 4 families total, and ATRUS with an identified HoxA11 mutation has been reported in at least 2 families. Since RUS may often be missed on routine physical examination, we recommend specific evaluation of pronation/supination in patients with hematological problems of unknown etiology. Additionally, we recommend that a targeted genetic panel be developed to detect mutations that are known for syndromes involving RUS, blood abnormalities, and other similar orthopedic entities that have cross-over manifestations like Thrombocytopenia-Absent Radii (TAR) Syndrome. This panel might consist of mutations associated with DBA (RPL5, RPL11, RPL35A, RPS7, RPS10, RPS17, RPS19, RPS24, and RPS26 mutations), with ATRUS (HoxA11 mutation), with IVIC Syndrome (SALL4 mutations), with Cohen Syndrome (8q22.2q22.3/COH1 deletion), with Noonan Syndrome (PTPN11, SOS1, RAF1, KRAS, NRAS, and BRAF mutations) and with TAR Syndrome (RBM8A null allele and noncoding SNP). The causative mutations of WT and RUS-H Syndromes have yet to be discovered. Genetic analysis of 6 of the 12 families with RUS-H Syndrome did not reveal a HoxA11 mutation. Broader sequencing techniques are underway for all 12 families in our RUS-H cohort, with hopeful detection of a new candidate gene as the unifying causative factor for the abnormalities in limb formation and hematopoiesis. Disclosures: Bussel: Ligand: Membership on an entity's Board of Directors or advisory committees, Research Funding; Immunomedics: Research Funding; IgG of America: Research Funding; Genzyme: Research Funding; GlaxoSmithKline: Family owns GSK stock, Family owns GSK stock Other, Membership on an entity's Board of Directors or advisory committees, Research Funding; Cangene: Research Funding; Amgen: Family owns Amgen stock Other, Membership on an entity's Board of Directors or advisory committees, Research Funding; Eisai: Membership on an entity's Board of Directors or advisory committees, Research Funding; Shionogi: Membership on an entity's Board of Directors or advisory committees, Research Funding; Sysmex: Research Funding; Portola: Consultancy.

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9

Tchervenkov, Christo I., Richard Tang, and Jeffrey P. Jacobs. "Hypoplastic Left Ventricle: Hypoplastic Left Heart Complex." World Journal for Pediatric and Congenital Heart Surgery 13, no. 5 (September 2022): 631–36. http://dx.doi.org/10.1177/21501351221116016.

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Hypoplastic left heart syndrome (HLHS) without intrinsic valvar stenosis or atresia is synonymous with the term hypoplastic left heart complex (HLHC) and is defined as a cardiac malformation at the milder end of the spectrum of HLHS with normally aligned great arteries without a common atrioventricular junction, characterized by underdevelopment of the left heart with significant hypoplasia of the left ventricle and hypoplasia of the aortic or mitral valve, or both valves, in the absence of intrinsic valvar stenosis or atresia, and with hypoplasia of the ascending aorta and aortic arch. This article describes the definitions, nomenclature, and classification of HLHC; the indications and contraindications for biventricular repair of HLHC; the surgical treatment of HLHC; and the associated outcomes.

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10

Morell, Victor O., James A. Quintessenza, and Jeffrey P. Jacobs. "Biventricular repair in the management of hypoplastic left heart syndrome." Cardiology in the Young 14, S1 (February 2004): 101–4. http://dx.doi.org/10.1017/s1047951104006390.

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Hypoplastic left heart syndrome is the term introduced by Noonan and Nadas1to describe a spectrum of cardiac anomalies characterized by varying degrees of significant underdevelopment of the left heart and aortic arch. These cardiac anomalies include mitral valvar disease, left ventricular hypoplasia, aortic stenosis at subvalvar, valvar, and supravalvar levels, hypoplasia of the ascending aorta and aortic arch, and aortic coarctation. Although descriptive, hypoplastic left heart syndrome suffers from being a very unspecific term, since it encompasses multiple degrees and combinations of abnormalities involving the left sided structures. In an attempt to provide gradings of severity, Kirklin and Barratt-Boyes2categorized these patients falling into the syndrome into four classes, according to whether obstruction was found at one, two, or more levels, or whether there is aortic atresia. Then, in 1998, Tchervenkov and colleagues3introduced the term hypoplastic left heart complex to describe a set of patients falling within the spectrum of hypoplasia of the left heart, but in the absence of intrinsic aortic or mitral valvar stenosis, this concept subsequently being endorsed by the International Committee established by the Society of Thoracic Surgeons, together with the European Association of Cardiothoracic Surgery, to rationalize the approach to nomenclature and databases.4The analysis offered by Tchervenkov et al.3showed that, in certain circumstances, there are potentially patients considered to have hypoplasia of the left heart who might be candidates for biventricular repair. If this is the case, then it is important to establish how this subset can be recognized, and how they are best treated.

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11

Soynov, Ilya, Alexander Omelchenko, Irina Keyl, Anastasiya Leykekhman, Oleg Chaschin, Meline Galstyan, Yuriy Gorbatykh, and Alexey Arkhipov. "Palliative surgery of a patient with hypoplastic left heart syndrome and low body weight." Journal of Experimental and Clinical Surgery 13, no. 1 (February 25, 2020): 51–54. http://dx.doi.org/10.18499/2070-478x-2020-13-1-51-54.

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Hypoplastic left heart syndrome is a congenital heart disease that affects the normal blood flow through the heart and it characterized by a critical underdevelopment of the left heart. Hypoplastic left heart syndrome is 1.43.8% among all congenital heart defects and 16% among critical congenital heart disease. Mortality in large cardiac surgery centers currently does not exceed 15%. However, mortality among patients with low body mass is up to 51% after the first stage of palliative surgery. In our clinical case, we describe hemodynamic surgery in neonatal with left-heart hypoplasia syndrome and low body weight (Norwood procedure with Sano shunt), postoperative case management inter-stage period and bidirectional cavopulmonary anastomosis procedure (second stage of hemodynamic correction).

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12

Quintessenza, James A., Victor O. Morell, and Jeffrey P. Jacobs. "Achieving a balance in the current approach to the surgical treatment of hypoplastic left heart syndrome." Cardiology in the Young 14, S1 (February 2004): 127–30. http://dx.doi.org/10.1017/s1047951104006456.

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In this Supplement, and at its associated Symposium “Controversies of Hypoplastic Left Heart Syndrome”, various contributors presented a variety of topics regarding “state of the art” treatments for hypoplastic left heart syndrome. The extent of the differences within these various presentations attests to the very significant challenges that remain in optimizing care of these patients, and related ones with other types of functionally univentricular hearts. Of these challenges, the initial surgical approach, be it staged palliation or cardiac transplantation, has been a hotly debated issue, with diametrically opposed schools of thought.1Today, with further experience, technical advances, and dramatically improved outcomes, there seems to be an emerging complimentary role for both options, favoring a staged reconstructive approach in the majority of circumstances. We review here the salient issues that have lead to this position, and discuss strategies for maintaining a program offering both staged palliation and transplantation for patients with hypoplasia of the left heart.

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13

Grossfeld, Paul D. "The genetics of hypoplastic left heart syndrome." Cardiology in the Young 9, no. 6 (November 1999): 627–32. http://dx.doi.org/10.1017/s1047951100005722.

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Hypoplastic left heart syndrome is one of the most therapeutically challenging congenital cardiac defects. It accounts for as many as 1.5% of all congenital heart defects, but is responsible for up to one quarter of deaths in neonates with heart disease.1The management of hypoplastic left heart syndrome is controversial. Two surgical options exist:2,3the Norwood procedure, is a three stage repair in which the morphologically right ventricle is converted to function as the systemic ventricle. Alternatively, orthotopic transplantation can be performed. Although both surgical options have had improved outcomes, the prognosis for long-term survival is guarded, with a five year survival for either approach reported to be in the region of 50–60%. In this review, I explore the evidence for a genetic etiology for the “classic” hypoplastic left heart syndrome, defined as mitral and/or aortic atresia with hypoplasia of the left ventricular cavity and the other left-sided structures.

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Jacobs, Jeffrey P. "Hypoplastic Left Ventricle: Definition, Morphology, and Classification of the Cardiac Phenotypes." World Journal for Pediatric and Congenital Heart Surgery 13, no. 5 (September 2022): 615–19. http://dx.doi.org/10.1177/21501351221114775.

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A prior manuscript provided information about hypoplastic left heart syndrome (HLHS) and related malformations, including definitions, morphology, and classification, based on the 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11). This manuscript provides information about the related cardiac phenotypes associated with “hypoplastic left ventricle” that might be suitable for biventricular repair, including definitions, morphology, and classification of the cardiac phenotypes of the following four congenital cardiac malformations that can all include a hypoplastic left ventricle: (1) hypoplastic left heart complex (HLHC), (2) critical aortic stenosis with left ventricular hypoplasia, (3) aortic atresia + ventricular septal defect, and (4) hypoplastic left ventricle with severely unbalanced atrioventricular septal defect.

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15

Ahmed, Md Khurshed, Harisul Hoque, SM Mustafa Zaman, Mukhlesur Rahman, Md Fakhrul Islam Khaled, MSI Tipu Chowdhury, Adnan Bashar, Md Ashraf Uddin Sultan, and Nilufar Fatema. "Holt-Oram Syndrome: A Variety of Heart-Hand Syndrome." University Heart Journal 13, no. 2 (July 26, 2018): 67–71. http://dx.doi.org/10.3329/uhj.v13i2.37666.

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Holt-Oram syndrome (HOS) is a heart-upper limb malformation complex , is a rare autosomal dominant disorder, caused by a mutation in the TBX5 gene located on chromosome 12 and near complete penetrance but variable expression. Holt and Oram first described this syndrome in 1960. Approximately 40% of cases represent new mutations. The syndrome is associated with defective development of cardiac structures that results in atrial septal defect(ASD), most commonly the secundum type; heart block of varying degree or both.The syndrome is characterized by cardiac malformations and aplasia or hypoplasia of the thumb. The incidence of HOS is estimated at 1:100,000 live births. In the literature, it is also known as atriodigital syndrome, heart-hand syndrome, upper limb-cardiovascular syndrome, cardiac-limb syndrome, or cardiomelic syndrome. Herein, we report a 45-year-old female case of HOS presenting a large ostium secundum type ASD along with congenitally fusion of carpal bones in both hands and absence of right first metacarpal bone with triphalagial thumb where as hypoplastic proximal phalange. In left hand, hypoplastic first metacarpal bone and hypoplastic thumb with syndactyly of both thumb with index finger.University Heart Journal Vol. 13, No. 2, July 2017; 67-71

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16

Borovikova, Ksenia Konstantinovna, Gulnur Kharisovna Shakirova, and Larisa Ivanovna Dolgikh. "A CASE OF PRENATAL ULTRASOUND DIAGNOSIS OF THE SYNDROM HYPOPLASTIC LEFT HEART." Scientific medical Bulletin of Ugra 27, no. 1 (2021): 50–53. http://dx.doi.org/10.25017/2306-1367-2021-27-1-50-53.

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The article describes the case of prenatal diagnosis: hypoplastic left heart syndrome in the period of pregnancy 31–32 weeks. In the postnatal period this syndrome have been verifi ed as Coarctation of the Aorta combined with Aortic Hypoplasia and abnormal development of the mitral and aortic valves.

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17

Rocha Martinez, Tania Leme da. "Successful Palliation in Monochorionic Twins with Hypoplastic Left Heart Syndrome." Cardiology Research and Reports 4, no. 4 (April 30, 2022): 01–02. http://dx.doi.org/10.31579/2692-9759/047.

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Hypoplastic left heart syndrome is a congenital heart disease which accounts for 8% of all congenital heart defects. Twin pregnancies have a significantly higher risk of structural heart disease than singleton ones, however the concordance rate has been found to be relatively low, even in monochorionic pregnancies. We describe here the favorable evolution of a monochorionic, diamniotic, twin pregnancy in which both fetuses were diagnosed with hypoplastic left heart syndrome by antenatal echocardiography. The present report suggests that at least in some cases there may be a genetic contribution to the etiology. The fetal diagnosis was imperative to a good postnatal outcome.

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18

deAlmeida, Angela, and David Sedmera. "Fibroblast Growth Factor-2 regulates proliferation of cardiac myocytes in normal and hypoplastic left ventricles in the developing chick." Cardiology in the Young 19, no. 2 (April 2009): 159–69. http://dx.doi.org/10.1017/s1047951109003552.

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AbstractThe developing heart increases its mass predominantly by increasing the number of contained cells through proliferation. We hypothesized that addition of fibroblast growth factor-2, a factor previously shown to stimulate division of the embryonic myocytes, to the left ventricular myocardium in an experimental model of left heart hypoplasia created in the chicken would attenuate phenotypic severity by increasing cellular proliferation. We have established an effective mode of delivery of fibroblast growth factor-2 to the chick embryonic left ventricular myocardium by using adenovirus vectors, which was more efficient and better tolerated than direct injection of recombinant fibroblast growth factor-2 protein. Injection of control adenovirus expressing green fluorescent protein did not result in significant alterations in myocytic proliferation or cell death compared with intact, uninjected, controls. Co-injection of adenoviruses expressing green fluorescent protein and fibroblast growth factor-2 was used for verification of positive injection, and induction of proliferation, respectively. Treatment of both normal and hypoplastic left ventricles with fibroblast growth factor-2 expressing adenovirus resulted in to 2 to 3-fold overexpression of fibroblast growth factor-2, as verified by immunostaining. An increase by 45% in myocytic proliferation was observed following injection of normal hearts, and an increase of 39% was observed in hypoplastic hearts. There was a significant increase in anti-myosin immunostaining in the hypoplastic, but not the normal hearts. We have shown, therefore, that expression of exogenous fibroblast growth factor-2 in the late embryonic heart can exert direct effects on cardiac myocytes, inducing both their proliferation and differentiation. These data suggest potential for a novel therapeutic option in selected cases of congenital cardiac disease, such as hypoplastic left heart syndrome.

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19

Cook, Amanda L., Christopher L. Curzon, and Angelo S. Milazzo. "An infant with hypoplastic left heart syndrome and spinal muscular atrophy." Cardiology in the Young 16, no. 1 (January 2006): 78–80. http://dx.doi.org/10.1017/s1047951105002131.

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We report an infant with hypoplastic left heart syndrome consisting of mitral valvar atresia, aortic valvar atresia, hypoplasia of the aortic arch and coarctation of the aorta, who demonstrated respiratory failure and global hypotonia, and who was eventually diagnosed with spinal muscular atrophy.

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20

Tchervenkov, Christo I. "Indications, criterions, and principles for biventricular repair." Cardiology in the Young 14, S1 (February 2004): 97–100. http://dx.doi.org/10.1017/s1047951104006389.

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The hypoplastic left heart syndrome constitutes a wide spectrum of cardiac malformations, characterized by varying degrees of underdevelopment of the structures of the left heart and aortic arch. At the severe end of the spectrum, there is aortic and mitral atresia, and the left ventricle is vestigial. In contrast, at the mild end, there is hypoplasia of the left ventricle in the absence of overt stenosis of either the aortic or mitral valves, the combination we have described as hypoplastic left heart complex.1 Until recently, debate with regard to the optimal surgical approach has centered on the choice between the Norwood operation and neonatal cardiac transplantation. In the last several years, nonetheless, it has been shown that of biventricular repair can be achieved in those patients at the favorable end of the spectrum that we designated as having the complex rather than the syndrome.1,2 It is well recognized that an ongoing difficulty is the inconsistent and imprecise definition of hypoplastic left heart syndrome. In this presentation, I summarize our own criterions, and our principles for biventricular repair, as based on our experience at the Montreal Children's Hospital, for those patients we judge to have the hypoplastic left heart complex.

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21

Grossfeld, Paul, Shuyi Nie, Lizhu Lin, Lu Wang, and Robert Anderson. "Hypoplastic Left Heart Syndrome: A New Paradigm for an Old Disease?" Journal of Cardiovascular Development and Disease 6, no. 1 (February 23, 2019): 10. http://dx.doi.org/10.3390/jcdd6010010.

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Hypoplastic left heart syndrome occurs in up to 3% of all infants born with congenital heart disease and is a leading cause of death in this population. Although there is strong evidence for a genetic component, a specific genetic cause is only known in a small subset of patients, consistent with a multifactorial etiology for the syndrome. There is controversy surrounding the mechanisms underlying the syndrome, which is likely due, in part, to the phenotypic variability of the disease. The most commonly held view is that the “decreased” growth of the left ventricle is due to a decreased flow during a critical period of ventricular development. Research has also been hindered by what has been, up until now, a lack of genetically engineered animal models that faithfully reproduce the human disease. There is a growing body of evidence, nonetheless, indicating that the hypoplasia of the left ventricle is due to a primary defect in ventricular development. In this review, we discuss the evidence demonstrating that, at least for a subset of cases, the chamber hypoplasia is the consequence of hyperplasia of the contained cardiomyocytes. In this regard, hypoplastic left heart syndrome could be viewed as a neonatal form of cardiomyopathy. We also discuss the role of the endocardium in the development of the ventricular hypoplasia, which may provide a mechanistic basis for how impaired flow to the developing ventricle leads to the anatomical changes seen in the syndrome.

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22

Anderson, Robert H., and Diane Spicer. "Fistulous communications with the coronary arteries in the setting of hypoplastic ventricles." Cardiology in the Young 20, S3 (December 2010): 86–91. http://dx.doi.org/10.1017/s1047951110001125.

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AbstractNeonates born with hypoplastic left heart syndrome now have a remarkably improved prognosis compared with the situation existing before the development of the Norwood sequence of operative procedures. Some of those born with hypoplastic right ventricles in the setting of pulmonary atresia with an intact ventricular septum, however, still have a relatively poor prognosis. In part this reflects the presence of fistulous communication between the cavity of the right hypoplastic right ventricle and the coronary arterial tree. Such fistulous communications are now increasingly recognised as being important in the setting of hypoplastic left heart syndrome. In this brief review, we describe the anatomy of the communications. Those found with hypoplastic right ventricles are seen most frequently when the cavity of the ventricle effectively represents only the inlet, this in turn reflecting mural overgrowth of the apical trabecular and outlet components during foetal development. This almost certainly reflects an earlier appearance of the pulmonary valvar lesion that promotes the cavitary hypoplasia. In those with hypoplastic left ventricles, the key feature differentiating those with fistulous communications is the presence of a patent mitral valve, since the left ventricle is typically no more than a virtual slit in postero-inferior ventricular wall in the setting of mitral valvar atresia or absence of the left atrioventricular connection.

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23

Somerset, David A., Katherine J. Barber, and Mark D. Kilby. "Outcomes following prenatal diagnosis of hypoplastic left heart syndrome." Fetal and Maternal Medicine Review 13, no. 3 (August 2002): 169–80. http://dx.doi.org/10.1017/s0965539502000323.

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Hypoplastic Left Heart Syndrome (HLHS) was first described fifty years ago. It is characterised by underdevelopment of the left ventricle with associated aortic and mitral valve hypoplasia or atresia, and varying degrees of hypoplasia of the aortic arch (Figure 1). In utero a physiological right to left shunt of oxygenated blood through the ductus arteriosus bypasses the obstruction and allows normal fetal growth. Closure of the ductus arteriosus in the post-natal period interrupts systemic blood flow, resulting in rapid deterioration and death. Untreated it is a universally fatal condition, leading to neonatal death within the first few days or weeks of life. Although HLHS affects only one baby in 10,000 and accounts for less than 10% of all congenital heart disease, HLHS is responsible for 25% of all deaths due to congenital cardiac disease occurring within the first week of life.

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24

Kaplinski, Michelle, and Meryl S. Cohen. "Characterising adequacy or inadequacy of the borderline left ventricle: what tools can we use?" Cardiology in the Young 25, no. 8 (December 2015): 1482–88. http://dx.doi.org/10.1017/s1047951115002267.

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AbstractBorderline left ventricle refers to a spectrum of left ventricular underdevelopment, typically associated with other cardiac anomalies. The left ventricle may be mildly hypoplastic, as is sometimes seen accompanying aortic coarctation, or it can be severely hypoplastic, as is seen in hypoplastic left heart syndrome. For patients with a borderline left ventricle that is at either extreme, the treatment decision is relatively straightforward. Those with the most severe form of left ventricle hypoplasia will require single ventricle palliation or cardiac transplantation, whereas those with the mildest form may not need any intervention. It is the management strategy of children that fall within the grey zone of the spectrum, which continues to be controversial and remains variable within and among different institutions. Cardiac diseases with associated left ventricle hypoplasia include critical aortic stenosis, mitral stenosis, coarctation of the aorta, arch hypoplasia, cor triatriatum, unbalanced common atrioventricular canal, Shone’s complex, total anomalous pulmonary venous return, and complex conotruncal abnormalities. In this review, we will discuss the assessment and management of infants with borderline left ventricle with critical aortic stenosis or arch obstruction and associated mitral anomalies.

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Weling, Rajaram M., and Nilima A. Narkhede. "Hypoplastic right heart syndrome." International Journal of Contemporary Pediatrics 9, no. 3 (February 23, 2022): 292. http://dx.doi.org/10.18203/2349-3291.ijcp20220458.

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Congenital heart diseases (CHDs) are of two types-acyanotic and cyanotic. Acyanotic heart diseases include atrial septal defect ASD, ventricular septal defect VSD, patent ductus arteriosus PDA, coarctation of aorta and atrioventricular cushion defects. Cyanotic heart disease includes Fallot’s tetralogy, transposition of the great vessels, Total anomalous pulmonary venous return, Tricuspid atresia and single ventricle or hypoplastic left heart or hypoplastic right heart syndrome (HRHS). Hypoplastic right heart is a rarer condition than the hypoplastic left heart. Our patient had a cyanotic heart disease; presented to us with hypoplastic right heart, a very rare association consisting of pulmonary atresia combined with a hypoplastic right ventricle and well-developed pulmonary arteries. Early clinical diagnosis and surgical intervention saved this child.

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DS, Akshatha, Shakuntala PN, Sreelatha S, Sumayya Sumayya, and Shruthi Shruthi. "A rare case of hypoplastic left heart syndrome in pregnancy and its outcome." New Indian Journal of OBGYN 8, no. 2 (February 2022): 321–23. http://dx.doi.org/10.21276/obgyn.2022.8.2.33.

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Gabriel, George C., Hisato Yagi, Xinxiu Xu, and Cecilia W. Lo. "Novel Insights into the Etiology, Genetics, and Embryology of Hypoplastic Left Heart Syndrome." World Journal for Pediatric and Congenital Heart Surgery 13, no. 5 (September 2022): 565–70. http://dx.doi.org/10.1177/21501351221102961.

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Hypoplastic left heart syndrome (HLHS) is a relatively rare severe congenital heart defect (CHD) closely linked to other left ventricular outflow tract (LVOT) lesions including bicuspid aortic valve (BAV), one of the most common heart defects. While HLHS, BAV, and other LVOT lesions have a strong genetic underpinning, their genetic etiology remains poorly understood. Findings from a large-scale mouse mutagenesis screen showed HLHS has a multigenic etiology and is genetically heterogenous, explaining difficulties in identifying the genetic causes of HLHS. In Ohia mice, HLHS shows incomplete penetrance. Some mice exhibited small LV with normal aorta, and others a normal LV with hypoplastic aorta, indicating the LV hypoplasia is not hemodynamically driven. In Ohia mutants, HLHS was found to have a digenic modular construction, with mutation in a chromatin modifier causing the small LV phenotype and mutation in Pcdha9 causing the aorta/aortic valve hypoplasia. The Pcdha9 mutation alone can cause BAV, and in the human genome two common deletion copy number variants spanning PCDHA7-10 are associated with BAV. Hence the digenic etiology of HLHS can account for the close association of HLHS, a rare CHD, with BAV, one of the most common CHD. Functional analysis of Ohia HLHS heart tissue showed severe mitochondrial dysfunction in the small LV, while the normal size RV is also affected but milder, suggesting possible role in vulnerability of surgically palliated HLHS patients to heart failure. These findings suggest insights into the genetics of HLHS may yield new therapies for improving outcome for patients with HLHS.

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Philip, Joseph, Dipankar Gupta, Mark S. Bleiweis, Biagio A. Pietra, and Himesh V. Vyas. "Hypoplastic left heart in Turner’s syndrome: a primary indication for transplant?" Cardiology in the Young 28, no. 3 (December 13, 2017): 458–60. http://dx.doi.org/10.1017/s1047951117001962.

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AbstractSurvival for hypoplastic left heart syndrome patients following the Norwood procedure is 71–90%. Mortality in patients with Turner’s syndrome and hypoplastic left heart syndrome after conventional palliation (Norwood operation) has been reported as high as 80%. This questions the approach of traditional staged palliation. Here, we report a patient with hypoplastic left heart syndrome and Turner’s syndrome bridged to orthotopic heart transplantation following a hybrid procedure.

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Newland, David P., Chin L. Poh, Diana Zannino, Patrick Elias, Christian P. Brizard, Kirsten Finucane, David S. Winlaw, and Yves d’Udekem. "The impact of morphological characteristics on late outcomes in patients born with hypoplastic left heart syndrome†." European Journal of Cardio-Thoracic Surgery 56, no. 3 (February 25, 2019): 557–63. http://dx.doi.org/10.1093/ejcts/ezz052.

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AbstractOBJECTIVESPatients with hypoplastic left heart syndrome are at high risk of late adverse events after Fontan completion, but it is unclear whether their morphological characteristics influence these outcomes.METHODSRetrospective review of the data from the Australian and New Zealand Fontan Registry identified 185 patients with hypoplastic left heart syndrome who survived to hospital discharge after Fontan completion. Their outcomes were reviewed to identify predictors of adverse events with a particular focus on the impact of morphological characteristics. All available echocardiographic parameters were collected, and the hypoplasia of the left ventricle was subjectively considered to be mild, moderate or severe.RESULTSThe mean follow-up after the Fontan procedure was 6.4 ± 4.7 years. The median age at Fontan procedure was 4.41 years, 95% (176/185) of patients underwent an extracardiac conduit Fontan procedure and 71% (132/185) of those were fenestrated. At 15 years after Fontan, freedom from death and cardiac transplantation was 90% [95% confidence interval (CI) 85–97], freedom from Fontan failure was 78% (95% CI 70–87) and freedom from adverse events was 32% (95% CI 22–46). Morphological parameters did not influence transplant-free survival or Fontan failure. Independent risk factors predicting higher incidence of adverse events included aortic atresia (P = 0.003).CONCLUSIONSThe long-term survival of Fontan survivors with hypoplastic left heart syndrome is excellent and appears comparable to that of the general Fontan population. However, intrinsic morphological characteristics may continue to burden patients with late morbidity.

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Jacobs, Jeffrey P. "Hypoplastic Left Heart Syndrome: Definition, Morphology, and Classification." World Journal for Pediatric and Congenital Heart Surgery 13, no. 5 (September 2022): 559–64. http://dx.doi.org/10.1177/21501351221114770.

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This manuscript will provide information about hypoplastic left heart syndrome (HLHS) and related malformations, including definitions, morphology, and classification, based on the 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11). HLHS is defined as “a spectrum of congenital cardiovascular malformations with normally aligned great arteries without a common atrioventricular junction, characterized by underdevelopment of the left heart with significant hypoplasia of the left ventricle including atresia, stenosis, or hypoplasia of the aortic or mitral valve, or both valves, and hypoplasia of the ascending aorta and aortic arch.” Functionally univentricular heart is defined as “a spectrum of congenital cardiac malformations in which the ventricular mass may not readily lend itself to partitioning that commits one ventricular pump to the systemic circulation, and another to the pulmonary circulation.” The Norwood operation is synonymous with the term “Norwood (Stage 1)” and is defined as (1) creation of an aortopulmonary connection and neoaortic arch construction resulting in univentricular physiology and (2) creation of a controlled source of pulmonary blood flow with a calibrated systemic-to-pulmonary artery shunt, a right ventricle to pulmonary artery conduit, or rarely, a cavopulmonary connection. The goals of the Norwood (Stage 1) Operation are creation of (1) unobstructed systemic blood flow via aortopulmonary connection and neoaortic arch construction, (2) unobstructed coronary blood flow, (3) unobstructed flow across the atrial septum, and (4) controlled pulmonary blood flow.

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Sahulee, Raj. "Surgical palliation for an infant with hypoplastic left heart syndrome and congenital lobar emphysema: a case report." Cardiology Research and Reports 3, no. 1 (March 16, 2021): 01–04. http://dx.doi.org/10.31579/2692-9759/015.

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Hypoplastic left heart syndrome is a form of congenital heart disease that is associated with significant lifelong morbidity and mortality. Although rarely reported together, the prognosis for patients with hypoplastic left heart syndrome and congenital anomalies of the airway or lung, such as congenital diaphragmatic hernia, has been shown to be very poor. The outcomes for patients with hypoplastic left heart syndrome and congenital lobar emphysema specifically is currently unknown. Reported herein is the first case of a child with a rare combination of hypoplastic left heart syndrome and congenital lobar emphysema, and a discussion of his presentation, diagnosis, management and outcome to date.

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Bondy, Carolyn A. "Hypoplastic Left Heart Syndrome." New England Journal of Medicine 362, no. 21 (May 27, 2010): 2026–28. http://dx.doi.org/10.1056/nejme1002923.

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33

Huhta, J. C., and W. I. Norwood. "Hypoplastic left heart syndrome." Current Opinion in Cardiology 3, no. 5 (September 1988): 781–84. http://dx.doi.org/10.1097/00001573-198809000-00022.

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KARL, HELEN W., FREDERICK A. HENSLEY, STEPHEN E. CYRAN, CARL A. FRANKEL, and JOHN L. MYERS. "Hypoplastic Left Heart Syndrome." Anesthesiology 72, no. 4 (April 1, 1990): 753–56. http://dx.doi.org/10.1097/00000542-199004000-00029.

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35

Stumper, O. "Hypoplastic left heart syndrome." Postgraduate Medical Journal 86, no. 1013 (March 1, 2010): 183–88. http://dx.doi.org/10.1136/hrt.2008.159889.

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36

TESTA, L., J. D. TOBIAS, and A. KAVANAUGH-MCHUGH. "Hypoplastic Left Heart Syndrome." Survey of Anesthesiology 39, no. 4 (August 1995): 212. http://dx.doi.org/10.1097/00132586-199508000-00003.

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37

Bailey, Leonard L., and Steven R. Gundry. "Hypoplastic Left Heart Syndrome." Pediatric Clinics of North America 37, no. 1 (February 1990): 137–50. http://dx.doi.org/10.1016/s0031-3955(16)36836-5.

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38

Gehrmann, Josef, Thomas Krasemann, Hans Gerd Kehl, and Johannes Vogt. "Hypoplastic Left-Heart Syndrome." Chest 120, no. 4 (October 2001): 1368–71. http://dx.doi.org/10.1378/chest.120.4.1368.

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39

Barron, David J., Mark D. Kilby, Ben Davies, John GC Wright, Timothy J. Jones, and William J. Brawn. "Hypoplastic left heart syndrome." Lancet 374, no. 9689 (August 2009): 551–64. http://dx.doi.org/10.1016/s0140-6736(09)60563-8.

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40

Rychik, Jack. "Hypoplastic Left Heart Syndrome." Circulation 130, no. 8 (August 19, 2014): 629–31. http://dx.doi.org/10.1161/circulationaha.114.011728.

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41

Sharland, Gurleen, Sherrida Rollings, John Simpson, and David Anderson. "Hypoplastic left-heart syndrome." Lancet 357, no. 9257 (March 2001): 722. http://dx.doi.org/10.1016/s0140-6736(05)71483-5.

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42

Hope, Peter, and Lindsey Allan. "Hypoplastic left heart syndrome." Lancet 337, no. 8753 (June 1991): 1349. http://dx.doi.org/10.1016/0140-6736(91)93022-2.

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Norwood, William I. "Hypoplastic Left Heart Syndrome." Cardiology Clinics 7, no. 2 (May 1989): 377–85. http://dx.doi.org/10.1016/s0733-8651(18)30442-9.

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44

Grossfeld, Paul D. "Hypoplastic Left Heart Syndrome." Journal of the American College of Cardiology 50, no. 16 (October 2007): 1596–97. http://dx.doi.org/10.1016/j.jacc.2007.06.045.

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45

Grossfeld, Paul, Maoqing Ye, and Richard Harvey. "Hypoplastic Left Heart Syndrome." Journal of the American College of Cardiology 53, no. 12 (March 2009): 1072–74. http://dx.doi.org/10.1016/j.jacc.2008.12.024.

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46

Bove, Edward L., and Amnon Rosenthal. "Hypoplastic left heart syndrome." Progress in Pediatric Cardiology 5, no. 1 (February 1996): 1–2. http://dx.doi.org/10.1016/1058-9813(95)00143-3.

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Norwood, William I. "Hypoplastic left heart syndrome." Annals of Thoracic Surgery 52, no. 3 (September 1991): 688–95. http://dx.doi.org/10.1016/0003-4975(91)90978-y.

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Clausen, Henning. "Hypoplastic left heart syndrome." Paediatrics and Child Health 25, no. 1 (January 2015): 18–22. http://dx.doi.org/10.1016/j.paed.2014.07.006.

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Mussa, Shafi, and David J. Barron. "Hypoplastic left heart syndrome." Paediatrics and Child Health 27, no. 2 (February 2017): 75–82. http://dx.doi.org/10.1016/j.paed.2016.12.002.

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Bardo, Dianna M. E., David G. Frankel, Kimberly E. Applegate, Daniel J. Murphy, and Russel P. Saneto. "Hypoplastic Left Heart Syndrome." RadioGraphics 21, no. 3 (May 2001): 705–17. http://dx.doi.org/10.1148/radiographics.21.3.g01ma09705.

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