Hypoplastic left heart syndrome: Progression of left ventricular dilation and dysfunction to left ventricular hypoplasia in utero

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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1212 LEFT VENTRICULAR AND CORONARY ARTERY MORPHOLOGY IN THE HYPOPLASTIC LEFT HEART SYNDROME

Pediatric Research ◽

10.1203/00006450-198104001-01238 ◽

1981 ◽

Vol 15 ◽

pp. 644-644

Author(s):

Willian O'Connor ◽

James Cash ◽

Carol Cottrill ◽

Gregory Johnson ◽

Jacqueline A Noonan

Keyword(s):

Coronary Artery ◽

Hypoplastic Left Heart Syndrome ◽

Left Ventricular ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Left Heart Syndrome

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Epidemiology of noncomplex left ventricular outflow tract obstruction malformations (aortic valve stenosis, coarctation of the aorta, hypoplastic left heart syndrome) in Texas, 1999-2001

Birth Defects Research Part A Clinical and Molecular Teratology ◽

10.1002/bdra.20169 ◽

2005 ◽

Vol 73 (8) ◽

pp. 555-561 ◽

Cited By ~ 52

Author(s):

Kim L. McBride ◽

Lisa Marengo ◽

Mark Canfield ◽

Peter Langlois ◽

David Fixler ◽

...

Keyword(s):

Aortic Valve ◽

Hypoplastic Left Heart Syndrome ◽

Left Ventricular Outflow Tract ◽

Coarctation Of The Aorta ◽

Ventricular Outflow Tract ◽

Left Ventricular ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Left Ventricular Outflow ◽

Left Heart Syndrome

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Hypoplastic Left Heart Syndrome: Color Doppler Sonographic and Magnetic Resonance Imaging Features in utero

Gynecologic and Obstetric Investigation ◽

10.1159/000292382 ◽

1995 ◽

Vol 39 (1) ◽

pp. 70-72 ◽

Cited By ~ 13

Author(s):

Kohkichi Hata ◽

Toshiyuki Hata ◽

Atsushi Manabe ◽

Manabu Kitao

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Hypoplastic Left Heart Syndrome ◽

Color Doppler ◽

In Utero ◽

Imaging Features ◽

Left Heart ◽

Resonance Imaging ◽

Hypoplastic Left Heart ◽

Left Heart Syndrome

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Assessment of Structural and Functional Abnormalities of the Myocardium and the Ascending Aorta in Fetus with Hypoplastic Left Heart Syndrome

BioMed Research International ◽

10.1155/2016/2616729 ◽

2016 ◽

Vol 2016 ◽

pp. 1-9

Author(s):

Yan Jiang ◽

Yali Xu ◽

Jinliang Tang ◽

Hongmei Xia

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Left Ventricular ◽

Ascending Aorta ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Histological Changes ◽

Left Heart Syndrome ◽

Rv Function ◽

Normal Controls ◽

Pulmonary Artery Diameter

Aims. To detect anatomical and intrinsic histopathological features of the ascending aorta and left ventricular (LV) myocardium and evaluate right ventricular (RV) function in fetuses with hypoplastic left heart syndrome (HLHS).Methods. Twenty-five fetuses diagnosed with HLHS were followed up in the antenatal and postpartum periods. 12 necropsy heart specimens were analyzed for morphological and histological changes.Results. Prenatal echocardiography and pathologic anatomy displayed the typical characteristics of HLHS as a severe underdevelopment of the LV in the form of mitral stenosis or atresia or as aortic atresia or stenosis, with a decreased ratio of aortic diameter to pulmonary artery diameter (median of 0.49 with a range of 0.24 to 0.69,p≤0.001) and a higher ratio of RV diameter to LV diameter (median of 2.44 with a range of 1.33 to 6.25,p≤0.001). The RV volume, stroke volume, and cardiac output in HLHS fetuses were increased compared with the gestational age-matched normal controls (p<0.01). Histological changes in the 12 HLHS specimens included LV myocardial fibrosis, aortic elastic fragmentation, and fibrosis.Conclusions. In addition to severe anatomical deformity, distinct histological abnormalities in the LV myocardium and aortic wall were identified in the fetuses with HLHS. RV function damage may be potentially exists.

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Improvement in right ventricular function by mitral valve closure in hypoplastic left heart syndrome

Interactive Cardiovascular and Thoracic Surgery ◽

10.1093/icvts/ivab037 ◽

2021 ◽

Author(s):

Dai Asada ◽

Yoko Kawai ◽

Yoshinobu Maeda ◽

Masaaki Yamagishi

Keyword(s):

Mitral Valve ◽

Right Ventricular ◽

Ventricular Function ◽

Hypoplastic Left Heart Syndrome ◽

Right Ventricular Function ◽

Left Ventricular ◽

Valve Closure ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Left Heart Syndrome

Abstract A male neonate presented with the aortic/mitral stenotic variant of hypoplastic left heart syndrome, wherein the suprasystemic left ventricular pressure and relatively large left ventricle had shifted the intraventricular septum. Despite bilateral pulmonary artery banding, the stroke volume was difficult to maintain owing to the compressed right ventricle, causing heart failure symptoms. Percutaneous balloon aortic valvuloplasty decreased the left ventricular pressure, restoring the right ventricular function. Norwood procedure with mitral valve closure after catheter intervention reduced the left ventricular size and improved the right ventricular function. This paper refers to the potential of mitral valve closure for hypoplastic left heart syndrome.

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