FIRST ATRIAL SEPTOSTOMY IN THE PHILIPPINES FOR IDIOPATHIC PULMONARY HYPERTENSION

Abstract

SESSION TITLE: Respiratory Care SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/10/2018 01:00 PM - 02:00 PM PURPOSE: Despite significant advances in pharmacological treatments, PAH remains to be an incurable disease with high morbidity and mortality. Although specific pharmacotherapies have shifted the survival curves of patients and improved exercise endurance as well as quality of life, it is also true that these pharmacological interventions are not always accessible and perhaps most importantly, not all these patients respond similarly to these drugs. Furthermore, many patients will continue to deteriorate and will eventually require an additional, non pharmacologic intervention such as Atrial Septostomy. METHODS: This is a case of a 38 year old female, known case of idiopathic pulmonary arterial hypertension diagnosed in 2007 through Right Heart Catheterization. She is currently maintained on Sildenafil, Bosentan, Furosemide, Amiodarone and Warfarin. Despite compliance to medications patient remained to be in Functional Class IV with PAP of 125mmHg. Patient also had recurrent hospitalizations due to dyspnea, chest pain and grade 3 bipedal edema. Hence patient was offered Atrial Septostomy as a treatment option. RESULTS: Hemodynamics post procedure showed a significant decrease in right atrial pressure to 10mmHg from 15mmHg and an increase in the left atrial pressure of 10mmhg from 8mmHg. The final size of the defect was 10mm. 2D echocardiogram done one year post Atrial Septostomy showed a Pulmonary Artery Pressure of 75mmHG which was the lowest pressure recorded in the course of her disease. Her functional Class also improved to II from IV. CONCLUSIONS: PAH remains a severe, life limiting condition, despite the introduction of multiple PAH specific therapies. Atrial Septostomy can provide hemodynamic and functional improvements and possibly survival benefits despite being previously known as only palliative or bridge to more definitive treatment for PAH. However with recent studies showing improved survival rates in severe PAH refractory to medical therapy, atrial septostomy may be life saving in our setting where lung transplantation is still unavailable.This is the first successfully performed atrial septostomy in the Philippines as a therapeutic option for severe, refractory end stage Pulmonary Arterial Hypertension. Although there are several areas for further study, our current thought is that this procedure is underutilized, which is probably related to overestimation of its risks and underestimation of its benefits. CLINICAL IMPLICATIONS: In view of poor prognosis and limited therapeutic options in our country, Atrial Septostomy becomes an alternative treatment option to patients with Pulmonary Hypertension. DISCLOSURES: No relevant relationships by Gracielle May Mendoza-Yu, source=Web Response