Pulmonary Hypertension in Congenital Heart Disease

Abstract

An important group of congenital cardiovascular defects is that characterized by the presence of an arterio-venous shunt, of which atrial septal defect, ventricular septal defect, and patent ductus arteriosus are common examples. In these the flow of blood is generally directed from left to right so that cyanosis is absent, but some may ultimately develop cyanosis (cyanose tardive) following a reversal of the shunt. Cardiac catheterization may then show the pressure in the pulmonary artery to be raised to the same level as that in the aorta.Ligature of a patent ductus is a safe procedure and the surgical treatment of atrial and ventricular septal defect is becoming established practice. The need now is to recognize in these cases the advent of pulmonary hypertension due to obstructive pulmonary vascular disease, for closure of the fistula in this circumstance cannot be curative.Although close attention has been paid to the pathology of pulmonary hypertension associated with congenital heart disease in recent years, there is need for further investigation into the mechanism of the hypertension, and a readier recognition of the particular congenital lesion associated with it. In previous papers we described pulmonary hypertension as a solitary finding (Evans et al., 1957), and again in association with mitral stenosis (Evans and Short, 1957). In this paper we deal with it in conjunction with congenital cardiovascular disease. Although such a combination is not rare in clinical practice, we have elected to describe here only the 11 cases that were ultimately examined at necropsy.As in our two previous series of patients a diagnosis of pulmonary hypertension was made from a clinical, electrocardiographic, and radiological examination, and independent of cardiac catheterization which was carried out in some of the patients. Thus, we have defined pulmonary hypertension as apersistent rise ofpulmonary arterial pressure sufficient to cause enlargement and ultimately failure of the right ventricle, with characteristic clinical, electrocardiographic and radiological signs. A perusal of the growing number of reports of pulmonary hypertension has convinced us of the need to adhere to this definition. The acceptance of pulmonary arterial pressure readings as the sole arbiter of pulmonary hypertension, irrespective of evidence of right ventricular hypertrophy, will lead inevitably to the confusion now commonplace in the diagnosis of systemic hypertension when deduced exclusively from manometric readings from the arm. In cases of congenital heart disease with left-to-right shunt the pulmonary arterial pressure is frequently raised, but in only a proportion of these is there true pulmonary hypertension. This distinction is imperative for the proper management of the patients.The 11 cases that we describe are arranged in four groups according to the kind of congenital anomaly found in association with pulmonary hypertension. Thus, there were three patients with atrial septal defect, three with ventricular septal defect, four wit...

Congenital Heart Disease and Pulmonary Hypertension

“…21 PAH is usually defined as the following: (1) mean pulmonary artery pressure of 25 mm Hg or greater and (2) either pulmonary capillary wedge pressure (PCWP) of 15 mm Hg or less or left ventricular end-diastolic pressure (LVEDP) of 15 mm Hg or less or measured left atrial pressure of less than 15 mm Hg and (3) PVR of 3 Wood units or greater. 22 It is inherent from these definitions that right heart catheterization is an essential test for the diagnosis of PAH.…”

“…1 Multiple studies have since expanded our understanding of the syndrome to include other congenital heart defects. [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] CHD is one of the world's leading birth defects and pulmonary arterial hypertension (PAH) associated with CHD is one of the most common causes of morbidity and mortality in this group of patients. 20 …”

Vorhofseptumdefekt und pulmonale Hypertonie

“…Dauer und Intensität des Hochdruckes bestimmen den Zeitpunkt, wann die Phase der funktionellen Adaptation in die der morphologisch faßbaren Veränderungen übergeht (SCHOENMACKERS [26]). EVANS [11] fandbei seinen Untersuchungen, daß die Veränderungen an den Gefäßwänden sich glichen, unabhängig davon, wo der Sitz des Defektes lag oder ob eine sog. essentielle pulmonale Hypertonie bestand.…”

Repeat Catheterization in Ventricular Septal Defect and Pulmonary Hypertension

“…Different opinions are held concerning the reversibility of the pulmonary hypertension in ventricular septal defect with a raised pulmonary vascular resistance. Thus Evans and Short (1958) thought it to be due to permanent structural changes, Burchell (1959) stated that acetylcholine caused a slight decrease in pressure in only 1 of 3 cases, Swan and Wood and Marshall, (1957) found that breathing 100per cent oxygen failed to produce a fall in pressure in some cases. Wood (1958) reported that acetylcholine did not produce a fall in pressure in the Eisenmenger syndrome.…”