Children born with HLHS are generally destined for one of two paths: heart transplant or the three-staged surgical procedures resulting in a Fontan circulation. For some children born with milder forms of HLHS, however, this may not be the case. In a recent report published in the Annals of Thoracic Surgery, Biventricular Repair After Stage II Univentricular Surgery: Palliation Is Not a One-Way Path, Drs. Muhammad Yasir Qureshi, Harold Burkhart, and Donald Hagler show that the left ventricle may continue to grow over time and, eventually, support a bi-ventricular circulation.
"For these patients, there is hope that such borderline left ventricles can continue to grow even without any specific intervention to promote its growth – which can ultimately result in a two-ventricle repair, avoiding all the nuisances and morbidities of single ventricle hemodynamics," said Dr. Qureshi, Advanced Cardiac Imaging Fellow at Mayo Clinic.
Compared to stage II or stage III palliation (Glenn and Fontan), there is no additional surgical risk to converting into a bi-ventricular circulation. These children still have smaller-than-normal left ventricles but there are no significant obstructions to systemic blood flow and the cardiac output is normal from the left heart.
Dr. Qureshi goes on to comment, "The decision to convert into two-ventricle repair is critical and needs detailed assessments of the left heart structures with several imaging modalities."
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The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of delaying or preventing heart failure for individuals affected by congenital heart defects including HLHS. The specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science, to diagnostic imaging to regenerative therapies.