scholarly journals Successful multimodality management of severe pulmonary arterial hypertension during pregnancy with VA-ECMO and atrial septostomy using stent

2019 ◽  
Vol 12 (12) ◽  
pp. e231916 ◽  
Author(s):  
Vorakamol Phoophiboon ◽  
Nattapong Jaimchariyatam ◽  
Suphot Srimahachota ◽  
Chayatat Sirinawin
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Early Period ◽  
Maximal Effect ◽  
Mechanical Support ◽  
Pulmonary Vasodilators ◽  
Atrial Septostomy ◽  
Pulmonary Arterial ◽  
Cardiac Circulation ◽  
Va Ecmo

A 30-year-old Thai woman (gravida 1, para 0) at 33 weeks gestation was referred to our hospital due to acute right ventricular failure. Pulmonary vasodilators were gradually administered before delivery. On the verge of sudden postpartum cardiac circulation collapse, she was resuscitated with veno-arterial extracorporeal membrane oxygenation (VA-ECMO). Abdominal compartment syndrome was developed in the early period of the mechanical support. Knowledge of pathophysiology about pulmonary arterial hypertension during pregnancy was applied. Atrial septostomy was the effective procedure for discontinuing mechanical support (VA-ECMO) corresponding to the suitable timing for maximal effect of pulmonary vasodilators. The patient and her child were safe and discharged in 2 months after the admission.

scholarly journals Interventional and Surgical Treatments for Pulmonary Arterial Hypertension

2021 ◽  
Vol 10 (15) ◽  
pp. 3326
Author(s):  
Tomasz Stącel ◽  
Magdalena Latos ◽  
Maciej Urlik ◽  
Mirosław Nęcki ◽  
Remigiusz Antończyk ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Interdisciplinary Approach ◽  
Left Ventricular ◽  
Free Survival ◽  
Atrial Septostomy ◽  
Surgical Options ◽  
Pulmonary Arterial ◽  
Va Ecmo ◽  
Surgical Treatments

Despite significant advancements in pharmacological treatment, interventional and surgical options are still viable treatments for patients with pulmonary arterial hypertension (PAH), particularly idiopathic PAH. Herein, we review the interventional and surgical treatments for PAH. Atrial septostomy and the Potts shunt can be useful bridging tools for lung transplantation (Ltx), which remains the final surgical treatment among patients who are refractory to any other kind of therapy. Veno-arterial extracorporeal membrane oxygenation (V-A ECMO) remains the ultimate bridging therapy for patients with severe PAH. More importantly, VA-ECMO plays a crucial role during Ltx and provides necessary left ventricular conditioning during the initial postoperative period. Pulmonary denervation may potentially be a new way to ensure better transplant-free survival among patients with the aforementioned disease. However, high-quality randomized controlled trials are needed. As established, obtaining the Eisenmenger physiology among patients with severe pulmonary hypertension by creating artificial defects is associated with improved survival. However, right-to-left shunting may be harmful after Ltx. Closure of the artificially created defects may carry some risk associated with cardiac surgery, especially among patients with Potts shunts. In conclusion, PAH requires an interdisciplinary approach using pharmacological, interventional, and surgical modalities.

Management of a child with pulmonary arterial hypertension presenting with systemic hypertension

2015 ◽  
Vol 26 (2) ◽  
pp. 378-381 ◽  
Author(s):  
Saul Flores ◽  
Joshua Daily ◽  
Jayant “Nick” Pratap ◽  
Michelle C. Cash ◽  
Russel Hirsch
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Systemic Hypertension ◽  
Clear Understanding ◽  
Successful Therapy ◽  
Compensatory Mechanisms ◽  
Pulmonary Vasodilators ◽  
Atrial Septostomy ◽  
Pulmonary Arterial ◽  
Severe Pulmonary Arterial Hypertension

AbstractWe describe the course and management of a 12-year-old girl with severe pulmonary arterial hypertension who initially presented with severe systemic hypertension. Successful therapy included pulmonary vasodilators and an atrial septostomy, while ensuring adequate maintenance of her systemic vascular resistance to maintain cardiac output. Clear understanding of the physiology and judicious medical management in patients with severe pulmonary arterial hypertension using extreme compensatory mechanisms is vitally important.

Central VA ECMO for Severe Pulmonary Arterial Hypertension: A Novel Cannulation Strategy

2018 ◽  
Vol 37 (4) ◽  
pp. S261-S262
Author(s):  
Y. Tipograf ◽  
B.A. Guenthart ◽  
A. Hozain ◽  
E. Berman Rosenzweig ◽  
M. Bacchetta
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Arterial ◽  
Va Ecmo ◽  
Severe Pulmonary Arterial Hypertension

scholarly journals 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

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.

scholarly journals Schistosomiasis Pulmonary Arterial Hypertension

2020 ◽  
Vol 11 ◽  
Author(s):  
Jean Pierre Sibomana ◽  
Aloma Campeche ◽  
Roberto J. Carvalho-Filho ◽  
Ricardo Amorim Correa ◽  
Helena Duani ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Heart Failure ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Systematic Screening ◽  
Pulmonary Vasodilators ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a disease of the lung blood vessels that results in right heart failure. PAH is thought to occur in about 5% to 10% of patients with hepatosplenic schistosomiasis, particularly due to S. mansoni. The lung blood vessel injury may result from a combination of embolization of eggs through portocaval shunts into the lungs causing localized Type 2 inflammatory response and vessel remodeling, triggering of autonomous pathology that becomes independent of the antigen, and high cardiac output as seen in portopulmonary hypertension. The condition is likely underdiagnosed as there is little systematic screening, and risk factors for developing PAH are not known. Screening is done by echocardiography, and formal diagnosis requires invasive right heart catheterization. Patients with Schistosoma-associated PAH show reduced functional capacity and can be treated with pulmonary vasodilators, which improves symptoms and may improve survival. There are animal models of this disease that might help in understanding disease pathogenesis and identify novel targets to screen and treatment. Pathogenic mechanisms include Type 2 immunity and activation and signaling in the TGF-β pathway. There are still major uncertainties regarding Schistosoma-associated PAH development, course and treatment.

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