Open Access

ARTICLE

Clinical findings in right ventricular noncompaction in hypoplastic left heart syndrome

by

Division of Pediatric Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

* Corresponding Author: Monique Gardner, Pediatrics, Division of Cardiology, The Children’s Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA 19104. Email:

Congenital Heart Disease 2017, 12(6), 783-786. https://doi.org/10.1111/chd.12506

Abstract

Background: Noncompaction is a poorly understood form of cardiomyopathy that typically affects the left ventricle and may be associated with congenital heart disease. Right ventricular noncompaction (RVNC) may occur when the left ventricle is affected but is rarely seen in isolation. RVNC may have clinical significance affecting surgical and long-term outcomes. We describe the diagnosis and clinical course in three patients at our institution.
Methods: We performed a retrospective review of patients diagnosed with RVNC over a 12- month period at our institution and reviewed their imaging and clinical course.
Results: Three patients were identified. All had diagnosis of RVNC by echocardiography (echo) made on postnatal imaging which reviewed degree of trabeculation, and noncompaction-tocompaction ratio of the myocardium. Patient A was a neonate with hypoplastic left heart syndrome (HLHS) who underwent a Norwood operation with Sano modification. Her postoperative course was notable for low-normal RV function. She returned with a pericardial effusion warranting immediate pericardiocentesis. She continued to have effusions, which were medically managed. She was subsequently found to have an RV apical pseudoaneurysm, which required surgical resection. Patient B was a neonate with HLHS who had a Norwood operation with Sano modification. She had low-normal RV function on echo. She required medical management for pericardial effusion. Patient C was a neonate with HLHS who also underwent a Norwood operation with Sano modification. His postoperative course was notable for elevated serum brain natriuretic peptide, which was treated with digoxin.
Conclusion: RVNC is a rare diagnosis with limited known clinical impact. One of these patients had a very rare complication after pericardiocentesis (pseudoaneurysm) that may have been related to the RVNC. Our understanding of this disease process is limited and requires additional investigation, but emphasizes the importance of appropriate diagnosis to allow for timely followup and counseling for this unique population.

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