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Pulmonary hypertension is present in approximately 5% of patients with adult congenital heart disease. These patients have worse functional status and increased mortality.
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There are various causes of pulmonary hypertension in patients with congenital heart disease. These causes include increased pulmonary blood flow, pulmonary vascular remodeling, and pulmonary venous hypertension. There is considerable overlap in patients with congenital heart disease.
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Effective treatment is not possible unless
Pulmonary Hypertension in Congenital Heart Disease: Beyond Eisenmenger Syndrome
Section snippets
Key points
Introduction and demographics
Approximately 3% to 10% of adults with congenital heart disease (ACHD) will develop pulmonary hypertension (PH).1, 2, 3 The causes of PH in congenital heart disease (CHD) are diverse. For example, patients with septal defects may have pulmonary arterial hypertension (PAH) with vascular remodeling and increased pulmonary vascular resistance (PVR), whereas patients with left-sided lesions may have PH due to pulmonary venous hypertension. In addition to different causes of PH associated with
Physical Examination and Bedside Tests Including 6-minute Walk Testing
The physical examination provides insight regarding the presence, severity, and consequences of PH in patients with CHD. Hepatomegaly, elevated jugular venous pressure, peripheral edema, ascites, and cool extremities are consistent with low-output right heart failure and suggest advanced disease. A pulsatile liver suggests substantial tricuspid regurgitation. Elevated jugular venous pressure is not specific for PAH and is present in many patients with PH due to left heart disease.
The right
Pulmonary Vascular Disease in Patients with Fontan Circulation
More than other forms of CHD, the Fontan circulation demands a low PVR. Because there is no subpulmonary ventricle in the Fontan circulation, chronically elevated central venous pressures drive blood through the lungs. If the transpulmonary gradient increases in the Fontan circulation, then central venous pressure must increase as well. Once the limited capacity to elevate central venous pressure is surpassed, cardiac output and ventricular preload will decrease. The inability to augment
Summary
ACHD-PH is a complex and evolving field. There are an increasing number of medical options, but optimal medication selection and overall therapeutic approach depend on understanding the underlying phenotype of the patient. Compared with non-ACHD patients with PH, the ACHD patient is more likely to have physiology explained by multiple causes/WHO groups, and therefore, requires an even more holistic approach to evaluation and management.
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Minimally Invasive Patent Ductus Arteriosus Ligation
2017, Clinics in PerinatologyCitation Excerpt :In persistently patent moderate or large defects, increased work of the left ventricle may lead to hypertrophy and ultimately failure.2,3 Persistently elevated pulmonary pressures due to long-standing pressure and volume overload can result in irreversible lung disease and pulmonary hypertension.3,9,10 Increased workload of the right ventricle to overcome elevated pulmonary pressures can result in hypertrophy of the right ventricle.3,9–11
Congenital Malformations Leading to Paradoxical Embolism
2016, Cardiology ClinicsCitation Excerpt :These functional and structural changes are eventually irreversible and the pulmonary vascular resistance exceeds that of the systemic vasculature with reversal of the shunt to be right to left. This progression of events is termed the Eisenmenger syndrome.41,42 The right-to-left shunt and consequent cyanosis and systemic hypoxia lead to a series of hematologic changes including erythrocytosis, hyperviscosity, and alterations in coagulability.33,35,43
Content validity of the nursing diagnostic Breathing Pattern, Ineffective, in children with congenital heart defects
2021, Revista Brasileira de EnfermagemNutritional status and risk factors for malnutrition in children with single ventricle physiology after total cavopulmonary connection
2018, Journal of Shanghai Jiaotong University (Medical Science)Idiopathic Pulmonary Arterial Hypertension in Children: A Review
2017, Pulmonary Therapy
Disclosures: Dr E.V. Krieger has received research grant funding from Actelion Pharmaceuticals US Inc. Dr P.J. Leary has nothing to disclose. Dr A.R. Opotowsky has received research grant funding from Actelion Pharmaceuticals US Inc and Merck & Co., Inc. Dr A.R. Opotowsky is supported by the Dunlevie Family Fund.