Dissecting aneurysm of the pulmonary arteries—an unusual complication of congenital heart disease

1994 ◽  
Vol 4 (2) ◽  
pp. 131-135
Author(s):  
Thomas M. Farrell ◽  
Carol M. Cottrill ◽  
William N. O'Connor ◽  
Dede Boucher ◽  
Jacqueline A. Noonan
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Clinical Presentation ◽  
Surgical Intervention ◽  
Pulmonary Arteries ◽  
Dissecting Aneurysm ◽  
Unusual Complication ◽  
Arterial Aneurysm ◽  
Pulmonary Arterial

SummaryDissection of a pulmonary arterial aneurysm due to underlying pulmonary hypertension from congenital heart disease is uniformly fatal, but fortunately rare. Two such cases are presented, along with review of 24 other known cases published in the literature. Clinical presentation, guidelines tomanagement, and possible surgical intervention in the acutely dissecting patient are discussed.

Pulmonary Arterial Hypertension In Adults With Congenital Heart Disease: General Overview of Disease Mechanisms

2007 ◽  
Vol 6 (3) ◽  
pp. 121-125 ◽  
Author(s):  
Ingram Schulze-Neick ◽  
John E. Deanfield
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Vascular System ◽  
Hypertensive Crisis ◽  
Multidisciplinary Care ◽  
Structural Heart Disease ◽  
Pulmonary Arterial

Adults with congenital heart disease (CHD) have become a rapidly expanding group of complex patients requiring multidisciplinary care in specialty centers by those trained in CHD. They represent one of the most challenging subgroups of patients with pulmonary arterial hypertension (PAH) due to the presence of structural heart disease with or without coexisting cyanosis and its complications. The primary focus of attention for these patients is the lungs, whose vascular system is affected by shunt flow, or is also congenitally malformed, or has been altered by surgical procedures. When PAH develops, it affects physical exercise tolerance, travel to high altitudes, pregnancy, operability, and anesthesia (myocardial failure due to pulmonary hypertensive crisis), and thus general morbidity and mortality in this special patient group.

scholarly journals Strategies for the management of pulmonary arterial hypertension in patients with congenital heart disease

2020 ◽  
Vol 4 (S1) ◽  
Author(s):  
Nathalie Liew ◽  
Zoya Rashid ◽  
Robert Tulloh
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Standard Management ◽  
Advanced Therapies ◽  
Pulmonary Arterial ◽  
The Uk

Abstract Background Pulmonary hypertension (PH) is commonly seen in adults who have congenital heart disease (CHD). Therapy is available for pulmonary arterial hypertension (PAH) and has greatly benefitted many patients with PAH related to CHD (PAH-CHD) over the last 15 years, with evidence of improved quality of life and prognosis in those with Eisenmenger syndrome and repaired PAH-CHD. In this review, we describe the standard management and advanced therapies for PAH, which are available in specialist PH centres around the UK and Ireland, and how these are used in PAH-CHD. Decisions around the choice of therapy are governed by commissioning and available evidence. Conclusion We explain the different pathways for action and the variety of medications now at our disposal to help this important group of patients.

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