The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales

Abstract

Background: Hypoplastic Left Heart Syndrome (HLHS) is a severe congenital heart defect (CHD) characterised by the underdevelopment of the left side of the heart with varying levels of hypoplasia of the left atrium, mitral valve, left ventricle, aortic valve and aortic arch. In the UK, age 12 survival for cases born between 1991 and 1993 was 21%. UK survival estimates corresponding to cases born between 2000 and 2015 were improved at 56%, but survival was examined up to age five only. Contemporary long-term survival estimates play a crucial role in counselling parents following diagnosis. The aim of this study was to report survival estimates up to age 15 for children born with HLHS or hypoplastic left ventricle with additional CHD in England and Wales between 1998 and 2012.Methods: Cases of HLHS notified to four congenital anomaly registers in England and Wales during 1998–2012, matched to Office for National Statistics mortality information, were included. Kaplan-Meier survival estimates to age 15 were reported. Cox regression models were fitted to examine risk factors for mortality.Results: There were 244 cases of HLHS and 99 cases of hypoplastic left ventricle co-occurring with other CHD, with traced survival status. Kaplan-Meier survival estimates for HLHS were 84.4% at age 1 week, 76.2% at 1 month, 63.5% at age 1 year, 58.6% at age 5 years, 54.6% at age 10 years, and 32.6% to age 15 years. The Kaplan-Meier survival estimates for cases of hypoplastic left ventricle co-occurring with additional CHD were 90.9% at age 1 week, 84.9% at 1 month, 73.7% at age 1 year, 67.7% to age 5 years, 59.2% to age 10 years, and 40.3% to age 15 years. Preterm birth (p = 0.007), low birth weight (p = 0.005), and female sex (p = 0.01) were associated with mortality.Conclusions: We have shown that prognosis associated with HLHS in the twenty first century exceeds that of many previous population-based studies, likely due to improvements in intensive care technologies and advances in surgical techniques over the last few decades.

Serial Assessment of Right Ventricular Deformation in Patients With Hypoplastic Left Heart Syndrome: A Cardiovascular Magnetic Resonance Feature Tracking Study

“… 2 The established surgical treatment strategy involves a 3‐stage palliation with creation of a total cavopulmonary connection (TCPC) being the third step. 3 Although survival rates for patients born in the 21st century compared with those born at the early 90s increased, 4 , 5 information about right ventricular (RV) function and deformation in patients with HLHS in Fontan circulation is still sparse. 6 , 7 …”

Flow-Mediated Factors in the Pathogenesis of Hypoplastic Left Heart Syndrome

“…Indeed, management of HLHS is largely limited to cardiac transplantation or a three-stage palliative surgical reconstruction procedure that is performed in early childhood [ 5 ]. These procedures are associated with significant morbidity (heart failure, arrhythmia, and hepatic dysfunction) [ 5 ], mortality (10-year survival rate ranges between approximately 40–55%) [ 6 , 7 , 8 ], and healthcare resources (median inpatient charges of approximately USD 376,403/child for surgical palliation and USD 582,920/child for cardiac transplantation) [ 9 ].…”

Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome

“…HLHS is characterized by severe underdevelopment of left sided heart structures including the mitral valve, left ventricle and aortic valve, resulting in a univentricular circulation. While single ventricle heart disease (SV) can be of either right ventricular (RV) morphology as in HLHS, or left ventricular (LV) morphology as in tricuspid or pulmonary atresia, patients with a systemic RV represent the most common SV sub-type with a prevalence of 2 to 3 per 10,000 live births worldwide [ 3 , 4 , 5 ]. In the United States alone, an estimated 1 in every 3841 infants is born with HLHS [ 6 ].…”

“…Without medical intervention, HLHS is responsible for 25–40% of all neonatal cardiac deaths [ 14 ]. The significant advancements of surgical palliation and cardiac transplantation have improved the 5-, 10-, and 15-year survival of patients by nearly 40% [ 3 , 15 , 16 , 17 ]. However, these interventions are not curative and life-long complications including pathological cardiac remodeling and progression to heart failure (HF) are still important consequences of HLHS [ 18 , 19 ].…”