Abstract
Atrial septostomy (AS) is recommended for pulmonary arterial hypertension (PAH)-associated right ventricular (RV) failure, recurrent syncope, or pulmonary hypertensive crisis (PHC). We aimed to evaluate the feasibility and efficacy of AS to manage PAH from infancy to adulthood. From June 2009 to December 2016, transcatheter atrial communications were created in 11 PAH patients (4 girls/women; median age = 4.3 years; range = 33 days–26 years; median body weight = 14 kg; range = 3–71 kg; NYHA-/Ross class IV; n = 11). PAH was classified as idiopathic (n = 6) or secondary (n = 5). History of syncope was dominant (n = 6); two with patent foramen ovale (PFO) admitted with recurrent PHC, three patients required resuscitation before AS. Three patients had PAH-associated low cardiac output. The average pulmonary arterial pressures (PAP systolic/diastolic) were 101/50 (±34/23); the corresponding systemic arterial pressures (SAP) were 99/54 (±23/11); and the mean ratio of PAPd / SAPd was 0.97 (±0.4). Percutaneous trans-septal puncture was uneventfully performed in nine patients; a PFO was dilated in two patients. There was no procedure-related mortality. The median balloon size was 10 mm (range = 6–14 mm); the mean catheter time was 174.6 ± 48 min; fluoroscopy time was 19.8 (±11) min. Syncope and PHC were successfully treated in all patients. The mean arterial oxygen saturation decreased from 97 ± 2 to 89 ± 11.7. One patient died awaiting lung transplantation, one continues to be listed; two patients received a reverse Potts-shunt, one patient died during follow-up; seven patients are stable with PAH-specific treatment. Percutaneous AS is an effective method palliating PAH-associated syncope, PHCs or right (bi-) ventricular heart failure.
Highlights
IntroductionFailure, recurrent syncope, or pulmonary hypertensive crisis (PHC), but only a few pediatric case series have been reported.[2,3] In adults, atrial septostomy (AS) is mostly performed as a bridge to lung transplant.[4] AS is used as an additional therapeutic strategy for pulmonary arterial hypertension (PAH)-associated right heart failure,[5] in cases where PAH-specific medical treatment fails[6,7] and as a palliative, pre-transplant therapy.[8,9] AS has been demonstrated in case series to improve survival of PAH patients[10] and to improve outcome, in particular if the approach is combined with PAH-specific treatment.[7] Lammers et al.[11] reported that until this date, the worldwide experience with AS was limited to approximately 280 adult and pediatric patients
The treatment of severe pulmonary arterial hypertension (PAH) remains challenging, despite improved medical options to address this life-threating condition
We present data on a relatively small number of patients, the present study confirms that generation of a restrictive atrial communication in PAH patients is a safe and effective approach, even as a high urgency procedure performed under resuscitation conditions
Summary
Failure, recurrent syncope, or pulmonary hypertensive crisis (PHC), but only a few pediatric case series have been reported.[2,3] In adults, AS is mostly performed as a bridge to lung transplant.[4] AS is used as an additional therapeutic strategy for PAH-associated right heart failure,[5] in cases where PAH-specific medical treatment fails[6,7] and as a palliative, pre-transplant therapy.[8,9] AS has been demonstrated in case series to improve survival of PAH patients[10] and to improve outcome, in particular if the approach is combined with PAH-specific treatment.[7] Lammers et al.[11] reported that until this date, the worldwide experience with AS was limited to approximately 280 adult and pediatric patients
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