Pushing the envelope: a treat and repair strategy for patients with advanced pulmonary hypertension associated with congenital heart disease

Abstract

Pulmonary arterial hypertension (PAH) is a frequent complication of congenital heart disease as a consequence of altered pulmonary hemodynamics with increased pulmonary blood flow and pressure. The development of pulmonary vascular disease (PVD) in this patient population is an important concern in determining operative strategy. Early, definitive surgical repair, when possible, is the best therapy to prevent and treat PVD. However, this is not possible in some patients because they either presented late, after the development of PVD, or they have complex lesions not amenable to one-step surgical correction, including patients with single ventricle physiology, who have a continuing risk of developing PVD. These patients represent an important, high-risk subgroup and many have been considered inoperable. We present a case series of two patients with complex congenital heart disease and advanced PVD who successfully underwent a treat and repair strategy with aggressive PAH therapies before surgical correction. Both patients had normalization of pulmonary vascular resistance prior to surgical correction. Caution is warranted in applying this strategy broadly and long-term follow-up for these patients is crucial. However, this treat and repair strategy may allow for favorable outcomes among some patients who previously had no therapeutic options.

Reverse Remodeling of Pulmonary Arterioles After Pulmonary Artery Banding in Patients ≥ 2 Years Old with Severe Pulmonary Arterial Hypertension and Congenital Heart Disease

“…Whether congenital heart disease combined with secondary severe pulmonary arterial hypertension can be cured depends on the age of the child, as well as the severity of preoperative pulmonary vascular lesions [14]. Nevertheless, it is still controversial whether the patients can safely undergo PAB and then single ventricular repair [3][4][5][6][7][8][9][10].…”

Pediatric ECMO Research: The Case for Collaboration

“…New generations of extracorporeal circuits are also being developed, including surface modifications with improved biocompatibility, miniaturized circuit or circuits with self-regulated flow-demand loops ( 35 – 37 ). Novel therapies for multimodal pulmonary hypertension management have the potential to alter the natural history of diseases in which ECMO may be considered ( 38 , 39 ). Innovative fetal therapies could similarly alter the natural history of diseases as varied as congenital diaphragmatic hernia, congenital heart disease, or congenital lung lesions ( 40 – 42 ).…”

Evolving Paradigms in the Treatment of Atrial Septal Defects With Pulmonary Arterial Hypertension

“…34,35 The "treat and repair" strategy in ASD-PAH has been gaining more traction over the years, initially with case reports and then followed by single-center data that support the role for interval PAHspecific therapy to achieve ASD closure criteria in patients who were previously deemed "non correctable." [36][37][38] In the ASD-PAH patient, the maximal hemodynamic response to PAH-specific therapy is typically demonstrated within 6 months of treatment. It is, therefore, reasonable to undertake repeat hemodynamic assessment after 6 months of optimized PAH medical therapy to make an initial assessment about candidacy for ASD closure.…”