Fenestrated ASD Closure in a Child with Idiopathic Pulmonary Hypertension and Exercise Desaturation

Abstract

Pulmonary arterial hypertension is a progressive disorder that may result in right heart failure and death. Atrial level shunts in the presence of pulmonary hypertension may allow right-to-left mixing with maintenance of cardiac output and improved survival. However, excessive mixing at the atrial level can cause undue systemic desaturation, increased fatigue and decreased exercise tolerance even in the presence of adequate cardiac output. A 5½-year-old was diagnosed with pulmonary hypertension, a large atrial septal defect and right-to-left shunting. Medical therapy over an 18-month period was successful in decreasing pulmonary artery pressure and pulmonary vascular resistance. However, because of the size and position of the intracardiac defect, symptoms of fatigue, and severe systemic desaturation with only minor activities persisted. Fenestrated surgical closure of the defect was thus undertaken to decrease the degree of atrial mixing, but still allow atrial decompression if necessary. Subsequent hemodynamic evaluation has demonstrated continued improvement, and all previous symptoms have resolved. Repeated echocardiography has confirmed patency of the atrial fenestration with left-to-right atrial flow.

Hemodynamic assessment of atrial septal defects

“…Successful closure of ASD following aggressive treatment with pulmonary vasodilators has been reported. Fenestrated closure of the defect to allow decompression of the right atrium may be an alternative in patients with more advanced disease (23,(28)(29)(30).…”

A novel method of creation of a fenestration in nitinol occluder devices used in closure of hypertensive patent arterial ducts

“…In patients with shunt lesions like atrial or VSDs or PDA with severe PAH, it is intuitive to either surgically band these hypertensive ducts or fenestrate the nitinol occluder devices used in cardiac catheter interventions. [ 8 9 10 11 12 ] Atrial septal occluder devices used in closure of secundum atrial septal defects are fenestrated either in factory (customized fenestrated devices, Occlutech, Helsingborg Sweden) or on table in the catheterization laboratory by tearing the fabric with surgical blades or electrical bipolar cautery perforation. [ 14 ] VSDs in hypertensive situations are also fenestrated in surgical theaters by fenestrating the patches or creating an unidirectional valve on the polytetrafluoroethylene patches.…”

“…[ 14 ] VSDs in hypertensive situations are also fenestrated in surgical theaters by fenestrating the patches or creating an unidirectional valve on the polytetrafluoroethylene patches. [ 7 8 9 10 11 ] Even though surgical banding of the hypertensive duct is a reliable way of reducing the duct size, it carries a risk of collapsing the left lung during the surgery that markedly worsens the borderline hemodynamics.…”

“…Prefabricated customized fenestrations and on table fenestrations have been utilized in nitinol atrial septal occluders in hypertensive atrial septal defects. [ 8 9 10 11 ] Surgical fenestrations of patches were also utilized for closing ventricular septal defects (VSDs) and they have also proven to be effective. [ 12 13 ] Fenestration of an occluder device or partial banding of the PDA may be a desirable strategy in very large hypertensive ducts with borderline operability.…”

Neonatal and Childhood Pulmonary Hypertension - An Update (2012)

“…Diagnosis may be delayed even when congenital heart disease is the cause of PAH. Early diagnosis is crucial as selected children with PAH-CHD may show a favorable response to targeted pulmonary hypertension drugs and may become eligible for surgical repair [62][63][64]. However, the "treat and repair" strategy remains to be validated, especially the long term results [65].…”