The Miracle Baby Grows Up: Hypoplastic Left Heart Syndrome in the Adult

Abstract

It has become increasingly clear that palliated adult single ventricle patients, like those with HLHS, face significant life-long morbidity from elevated systemic venous pressures as a consequence of the Fontan procedure. Downstream organ dysfunction secondary to elevated Fontan pressures has the potential to significantly impact long-term management decisions, including strategies of organ allocation. Because of the presence of a morphologic systemic right ventricle, HLHS patients may be at even higher risk than other adult patients with a Fontan. Because the adult HLHS population continues to grow, recognition of common sources of patient morbidity and mortality is becoming increasingly important. A coordinated effort between patients and providers is necessary to address the many remaining areas of clinical uncertainty to help ensure continued improvement in patient prognosis and quality of life.

Medical and Day-to-Day Burden on Families of Children with Hypoplastic Left Heart Syndrome Who Have Undergone Single Ventricle Surgical Palliation over a Decade

“…It is imperative that families understand and appreciate what these surgeries entail and what life after SVSP may look like when they choose surgical palliation [11]. Even with improving survival, children with HLHS need continuous medical attention and face frequent complications if they are to survive to adulthood [4,12,13]. After hospital discharge, they require daily medications, frequent doctors' appointments and further invasive procedures and testing [14].…”

Comparative Metabolomics in Single Ventricle Patients after Fontan Palliation: A Strong Case for a Targeted Metabolic Therapy

“…Even if the outcome of SV patients is steadily improving, particularly their largest subgroup—i.e., SV patients with aortic atresia and a hypoplastic left heart (HLHS, 40% of cases [ 5 ]) and a morphologically right ventricle (RV) serving as the subsystemic ventricle—performs worse than patients with a morphologically left ventricle (LV) [ 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 ], particularly with respect to atrioventricular valve failure, impaired ventricular function and/or failure of the Fontan circulation with upstream issues such as liver cirrhosis or protein-losing enteropathy [ 15 , 16 , 17 ]. Ventricular dysfunction is currently considered to be inevitable for SV patients [ 18 ].…”