Background: Potts shunt has been suggested as an effective palliative therapy for patients with pulmonary artery hypertension (PAH) not associated with congenital heart disease.
Materials And Methods: This is a prospective single-center study performed to assess outcomes of Potts shunt in patients with PAH who are in functional class III or IV.
Results: 52 patients in functional class III/IV with pulmonary arterial hypertension without significant intra or extracardiac shunt on maximal medical therapy were evaluated and counseled for undergoing Potts shunt/patent ductus arteriosus (PDA) stenting. 16/52 patients (13 females) consented for the procedure; 14 patients underwent surgical creation of Potts, and 2 underwent transcatheter stenting of PDA, which physiologically acted like a Potts shunt. Standard medical therapy was continued in patients who did not consent for the procedure. 12/16 patients survived the procedure. Patients who did not survive the procedure were older, with severe right ventricular systolic dysfunction, and functional class IV. Patients who survived the procedure were followed up in the pulmonary hypertension clinic. The Median follow-up was 17 months (1-40 months). 11/13 patients discharged after the operation showed sustained clinical, echocardiographic, and biochemical improvement, which reduced need for pulmonary vasodilator therapy in 10/13 patients. There was one death in the follow-up period 16 months post-surgery due to lower respiratory tract infection.
Conclusion: Potts shunt is feasible in patients with PAH without significant intra or extracardiac shunts. It can be done safely with an acceptable success rate. Patient selection, preoperative stabilization, and meticulous postoperative management are essential. It should be performed at the earliest sign of clinical, echocardiographic, or laboratory deterioation for optimal outcomes. Long-term follow-up is required to see a sustained improvement in functional class and the need for a lung transplant in the future.
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http://dx.doi.org/10.1016/j.ihj.2021.01.007 | DOI Listing |
Pulm Circ
October 2024
UK Service for Pulmonary Hypertension in Children, Great Ormond Street Hospital for Children London UK.
The aim of this single-centre retrospective observational study was to evaluate the safety, tolerability, and efficacy of an in-class combination therapy switch from bosentan plus sildenafil to ambrisentan plus tadalafil in children with pulmonary arterial hypertension. Children aged over 5 years who were established on sildenafil plus bosentan were offered to undergo a therapy switch from May 2014 to May 2021 and, if remaining in the service, followed up to May 2024. Children with Eisenmenger syndrome, open intra or extra-cardiac shunt, or with pulmonary hypertension-associated lung disease were excluded.
View Article and Find Full Text PDFAm J Respir Crit Care Med
November 2024
Cincinnati Children's Hospital Medical Center, Cardiothoracic Surgery, Cincinnati, Ohio, United States.
Background: Pulmonary hypertension in children often progresses despite optimal therapy. This document provides an evidence-based clinical practice guideline for the management of children with progressive pulmonary hypertension despite optimal therapy.
Methods: A multidisciplinary panel identified pertinent questions regarding the management of children with pulmonary hypertension that has progressed despite optimal therapy, conducted systematic reviews of the relevant literature, and applied the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach to develop clinical recommendations.
Neurosurgery
October 2024
Department of Neurosurgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Background And Objectives: The most common treatment of hydrocephalus is ventriculoperitoneal (VP) shunting. Peritoneal access is commonly conducted through an open laparotomy, but laparoscopic peritoneal access is gaining popularity. Many studies have reported the benefits of minimally invasive laparoscopic peritoneal access, but there is no consensus on its use.
View Article and Find Full Text PDFEur Respir J
October 2024
Center for Congenital Heart Diseases, Paediatric and Congenital Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Paediatric pulmonary arterial hypertension (PAH) shares common features with adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for caring for infants and children with PAH, as presented by the paediatric task force of the 7th World Symposium on Pulmonary Hypertension. We provide updates on diagnosing, classifying, risk-stratifying and treating paediatric pulmonary hypertension (PH) and identify critical knowledge gaps.
View Article and Find Full Text PDFPediatr Cardiol
April 2024
Pediatric Cardiology, University Clinic Frankfurt, Frankfurt, Germany.
Arterial duct stenting, pioneered in the early 1990s for newborns with a duct-dependent pulmonary and systemic circulation, has evolved significantly over the past decades. This progressive technique has led to the development of novel therapeutic strategies, including the Hybrid approach introduced three decades ago, and more recently, a complete transcatheter approach for treating newborns with hypoplastic left heart syndrome (HLHS). Subsequently, the transcatheter method has been extended to bi-ventricular lesions and patients with pulmonary hypertension, establishing a reverse Potts-shunt pathophysiology.
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