First-in-man experience with cryoplasty during graded balloon atrial septostomy to reduce spontaneous closure in a patient with severe pulmonary arterial hypertension

Pulmonary arterial hypertension is a pernicious disease with a diverse etiology in the pediatric population. Despite the increased availability of drug therapies, pulmonary arterial hypertension continues to cause significant morbidity and mortality. In pediatric patients with severe pulmonary arterial hypertension who have failed medical therapy, a few studies have demonstrated the role of balloon atrial septostomy as a bridge to lung transplantation or a means of improving symptomatology. However, no data exists on the utilization of balloon atrial septostomy as a palliative intervention to wean from extracorporeal membrane oxygenation (ECMO) when all other therapies are exhausted. Here we describe a case series of two pediatric patients with severe pulmonary arterial hypertension, requiring ECMO support, who were successfully weaned from ECMO following balloon atrial septostomy.

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Atrial septostomy in the treatment of severe pulmonary arterial hypertension

Heart ◽

10.1136/heart.90.1.69 ◽

2004 ◽

Vol 90 (1) ◽

pp. 69-69

Author(s):

F Reichenberger

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Atrial Septostomy ◽

Pulmonary Arterial ◽

Severe Pulmonary Arterial Hypertension

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Balloon atrial septostomy and transition of subcutaneous to intravenous prostacyclin infusion for rescuing advanced right heart failure in idiopathic pulmonary arterial hypertension: a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytaa052 ◽

2020 ◽

Vol 4 (3) ◽

pp. 1-5

Author(s):

Kae-Woei Liang ◽

Kuo-Yang Wang

Keyword(s):

Heart Failure ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Right Heart Failure ◽

Idiopathic Pulmonary Arterial Hypertension ◽

Right Heart ◽

Balloon Atrial Septostomy ◽

Atrial Septostomy ◽

Pulmonary Arterial ◽

Prostacyclin Analogues

Abstract Background Intravenous (IV) prostacyclin analogues infusion and balloon atrial septostomy (BAS) are two important treatment options for managing advanced right heart failure in patients with idiopathic pulmonary arterial hypertension (IPAH). References and protocols are rare for dose titrations and transitions between subcutaneous and IV prostacyclin in functional Class IV IPAH patients. Balloon atrial septostomy is rarely done in very few expert centres. Case summary A young female with IPAH who had received maximal medication including subcutaneous prostacyclin analogues injection was admitted due to advanced right heart failure. She received ascites drainage twice. Later, we directly switched the administration route of prostacyclin from subcutaneous to IV at a ratio of 1:1 instantly. Such rapid conversion led her into a state of profound hypotension and drowsy consciousness, which was resolved after escalating IV inotropics and reducing prostacyclin dosage. Five days later, she received BAS under the guidance of intracardiac echocardiography. Her urine output increased and dyspnoea improved gradually. Six months later, clinical worsening happened again with increase of ascites and dyspnoea. She underwent 2nd and 3rd session of graded BAS with relief of symptoms again. She received permanent transition to IV prostacyclin analogues infusions via a peripherally inserted central catheter after three sessions of BAS. Discussion Balloon atrial septostomy is effective in stabilizing the critical right heart failure in IPAH patients but should be intended as a bridge to lung transplant procedure. Transition from subcutaneous to IV prostacyclin is helpful but needs to be titrated in proper aliquots and time intervals to avoid abrupt haemodynamic changes.

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