Dysplastic pulmonary valve complicates HLHS palliation

The patient is a newborn with hypoplastic left heart syndrome (HLHS) with a dysplastic pulmonary valve and progressive pulmonary valve stenosis.

The patient

Newborn with hypoplastic left heart syndrome (HLHS) with a dysplastic pulmonary valve and progressive pulmonary valve stenosis

The challenge

The dysplastic pulmonary valve makes it unsuitable to proceed with the typical first stage of single ventricle palliation for hypoplastic left heart syndrome (HLHS) — the Norwood procedure, which would transition the pulmonary valve into the neo-aortic valve. While pulmonary valve stenosis that would subsequently lead to neo-aortic valve outflow obstruction is rare and not well described in the literature,  it is typically considered a contraindication for a stage 1 Norwood procedure.

The decision on how to proceed came in two parts: First, can the patient transition down the standard single ventricle pathway or do we move forward with heart transplant? And if transplant is deemed the best option, how do we palliate his physiology and successfully bridge to heart transplant?

Echocardiogram image of subcostal view of the right ventricle and the pulmonary valve. The pulmonary valve appears thickened and mildly dysplastic.

Echocardiogram images with side-by-side standard and color images of an apical view of the right ventricular outflow tract. There is flow acceleration starting at the level of the pulmonary valve indicating pulmonary valve stenosis.

The providers

All are with Norton Children’s Heart Institute, affiliated with the UofL School of Medicine

Bahaaldin Alsoufi, M.D., chief of pediatric cardiothoracic surgery and co-director of Norton Children’s Heart Institute

Andrea Lambert, M.D., pediatric cardiologist

Sarah Jane Wilkens, M.D. MPH, pediatric cardiologist

The solution

The decision was made to list the patient for heart transplant. Given that the transplant wait list duration for his age group until a suitable donor heart becomes available is expected to be up to six months, a decision was made to palliate him via cardiac surgery to have bilateral pulmonary artery bands placed to prevent pulmonary overcirculation, while his patent ductus arteriosus (PDA) was maintained open on prostaglandin (PGE) infusion. He then ultimately underwent cardiac catheterization to have a PDA stent placed to maintain ductal patency and support his systemic circulation. He recovered well from the procedures and was maintained in the hospital while waiting for a new heart. During the majority of his hospitalization he did not require respiratory support and was on full enteral feeds. He was able to progress in developmental milestones while waiting on the heart transplant list.

The result

A dysplastic pulmonary valve in the setting of HLHS is very rare. It has been reported in the literature in only a handful of case reports over the last 30 years. Most commonly, patients with this constellation of heart defects have not survived prior to heart transplantation. Fortunately, this patient underwent a successful heart transplant and now has good graft function. The patient was able to be discharged home from the hospital and continues to do well.

Treatment and results may not be representative of all similar cases.

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To refer a patient to Norton Children’s Heart Institute, visit Norton EpicLink and choose EpicLink referral to Pediatric Cardiothoracic Surgery.

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