Decompressive Atrioseptostomy (DAS) for the Treatment of Severe Pulmonary Hypertension Secondary to Restrictive Cardiomyopathy

Objective: Restrictive cardiomyopathy of the left ventricle is characterized by severely impaired diastolic function leading to subsequent left atrial and pulmonary congestion and secondary elevation of pulmonary artery pressures (PAP) and pulmonary vascular resistance (PVR). Treatment options are rare, cardiac transplantation (CTX) may be necessary, elevated PVR however excludes patients from CTX and assist device implantation may be required as bridge to transplant modality. Decompression of the left atrium by creation of a small atrial septal defect (ASD) has proven to be effective in pulmonary congestion after severe systolic heart failure in ECMO patients. We describe our first patient in whom creation of a small ASD as decompressive atrioseptostomy (DAS) significantly reduced elevated PVR.

Method/Result: A 13-year-old girl presented in NYHA II-III caused by severe diastolic dysfunction in restrictive non compaction cardiomyopathy. Severe pulmonary hypertension secondary to the underlying physiology excluded her from enrolment and listing for cardiac transplantation. Decompressive balloon atrioseptostomy was performed after transseptal puncture and static septum dilatation using a 12 mm balloon creating a small ASD with left-to-right shunting. There was immediate decompression of the left atrium with reduction of the left atrial pressure and subsequent significant decline of pulmonary artery pressure and pulmonary vascular resistance without impact on systemic cardiac output persistent now after 3 weeks and 4 months. The patient improved clinically and now is in NYHA class I; Based on the positive result, listing for transplantation could be postponed.

Conclusion: Severe diastolic dysfunction of the left ventricle may cause secondary left atrial and pulmonary congestion and subsequent elevation of pulmonary vascular pressure and resistance. The creation of a small atrial septal defect can effectively decompress the left atrium, reduce the left atrial pressure and subsequently the pulmonary congestion without reducing cardiac output. DAS seems to be a safe and easy concept to treat this underlying pathophysiology and reduce secondary pulmonary effects of diastolic dysfunction and enable enrolment for CTX.