Donald Heath

Pulmonary Veno-Occlusive Disease

A woman, 45 years old at the time of her death, showed the clinical features of primary pulmonary hypertension and the results of cardiac catheterization and pulmonary function tests were consistent with this diagnosis. Histological examination of the lung showed that the pulmonary veins were severely involved by cellular fibrous tissue. These distinctive lesions appear to constitute a separate disease entity for which the name “pulmonary veno-occlusive disease” is suggested.

Pulmonary hypertension due to myxoma of the right atrium

Abstract A 35-year-old woman developed a pulmonary arterial hypertension of 52 25 mm. Hg , with a diastolic pressure gradient of 10 mm. Hg across the tricuspid valve. Radiography revealed shadowing in the right lower lobe, and angiocardiography showed an enormous filling defect in the right atrium. A diagnosis of pulmonary hypertension with underlying obstructive pulmonary vascular disease complicating a myxoma of the right atrium was made successfully, and the mass was removed surgically. The patient died of congestive cardiac failure after the operation. The pulmonary arteries were found to be occluded by emboli of myoxoma which had actively infiltrated the media. The appearances were totally unlike those of recurrent pulmonary thromboembolism. This capacity for limited invasiveness leads us to believe that the cardiac myxoma is a true neoplasm and not a degenerate thrombus.

PATENT DUCTUS ARTERIOSUS WITH PULMONARY HYPERTENSION

In most cases of patent ductus arteriosus the diagnosis is readily made on recognition of the typical murmur. In some, however, the continuous murmur is lacking, and a systolic murmur only, of variable loudness, is heard at the pulmonary area. This is not uncommon in infants and young children, who may later develop the characteristic murmur (Gilchrist, 1945). Occasionally the continuous murmur is absent in adults in whom patency of the ductus is found subsequently at autopsy: in such cases the state of the pulmonary arteries and the presence of right ventricular hypertrophy may indicate that the pulmonary arterial pressure was much raised in life (Holman, 1925; Keys and Shapiro, 1943 (Case 3); Chapman, 1944; Douglas et al., 1947; Ulrich, 1947). As a result, the pressure gradient between the aorta and the pulmonary artery may be so diminished that the blood flow along the ductus is insufficient to set up a continuous murmur. In extreme examples the pressure in the pulmonary artery may exceed that in the aorta, causing a reversal of the usual left to right shunt (Johnson et al., 1950; Campbell and Hudson, 1951; Dammann et al., 1953). Three cases of patent ductus arteriosus complicated by severe pulmonary hypertension are presented here. The diagnosis was made in each on cardiac catheterization. In two, pulmonary hypertension caused reversal of the shunt; in the third the shunt was not reversed, but there was, in addition, coarctation of the aorta.

Hypertensive Pulmonary Vascular Disease

Patients with pulmonary artery blood pressures that are chronically elevated to levels of the order of the systemic blood pressures, form a distinct clinicopathologic entity. This condition may complicate a ventricular septal defect, Eisenmengers complex, patent ductus arteriosus, atrial septal defect, and mitral stenosis, and it may occur as idiopathic pulmonary hypertension. The clinical picture is dominated by symptoms and signs characteristic of pulmonary hypertension, which often mask underlying cardiac anomalies, and definitive pathologic changes occur in the pulmonary vasculature. The term hypertensive pulmonary vascular disease is therefore suggested for this distinct group of patients.

Idiopathic pulmonary hypertension.

Since it became possible to record pulmonary artery blood pressures directly by cardiac catheterization, several authors have described severe pulmonary arterial hypertension in patients without any apparent predisposing cause such as a septal defect or mitral stenosis. The purpose of the present communication is to describe four further patients with idiopathic pulmonary hypertension and to emphasize that the clinical features and pathological lesions in the lungs of these cases are similar to those occurring in other patients with severe pulmonary hypertension secondary to congenital septal defects. Heath and Whitaker (1956) have pointed out that all patients with severe pulmonary hypertension present a distinct clinico-pathological entity, which they term hypertensive pulmonary vascular disease; it is dominated in life by the symptoms and signs of pulmonary hypertension and characterized at autopsy by specific changes in the small pulmonary blood vessels.

Pathology of the lungs in mitral stenosis in relation to respiratory function and pulmonary haemodynamics.

In mitral stenosis, chronic rises in left atrial pressure lead to progressive changes in the structure and function of the lungs: these have been reviewed by Arnott in his Croonian lectures of 1963. We have investigated a small group of patients with this disease in order to study the relation between histological changes in their lungs and abnormalities in their pulmonary heemodynamics and respiratory function.

Cardioaortic Fistula A Case Diagnosed in Life and Treated Surgically

A case of cardioaortic fistula, due to rupture of an aneurysm of a sinus of Valsalva into the right ventricle, is described. This case was diagnosed in life and treated surgically one year later when the patient was admitted to hospital with bacterial endocarditis. The clinical features are discussed and the role of special methods of investigation in the diagnosis of the condition is defined. The importance of cardiac catheterization is stressed. The operative technique is described briefly. Following the patients death from the uncontrolled infection an autopsy examination was carried out and the findings in the heart and small pulmonary vessels are given.

The small pulmonary blood vessels in atrial septal defect.

The development of direct methods of recording pulmonary artery pressure by cardiac catheterization or bronchoscopically and the advance of cardiac surgery have stimulated an interest in pulmonary hypertension and have made it possible to study the relation of morbid anatomical and dynamic changes in the pulmonary circulation. In recent years there have been many reports of abnormal pulmonary vessels in patients with pulmonary hypertension and these suggest that there is a histological pattern characteristic of this disorder. Although pulmonary artery blood pressures may range from normal to levels of the order of the systemic blood pressure in patients with atrial septal defects there have been but few descriptions of lesions in the small pulmonary blood vessels in this anomaly (Welch and Kinney, 1948; and Evans, 1951) and an examination of the pulmonary vascular histology in a group of such cases seemed likely to provide further evidence of anatomical changes occurring with pulmonary hypertension. In the present investigation the pulmonary blood vessels from a small group of patients with atrial septal defects, where the pulmonary artery blood pressures were known to range from normal to severe pulmonary hypertension, were examined histologically. The anatomical changes were related to the pulmonary artery blood pressures and there appeared to be well defined histological pictures characteristic of normal and of severely raised pulmonary arterial pressure. Method. Specimens of lung were obtained at autopsy from five patients with atrial defect whose pulmonary artery pressures had been previously recorded by cardiac catheterization. Four died following attempts to close the septal defects while the other (Case 3) died from a paroxysm of tachycardia. Sections of lung from all cases were stained to show elastic by the Lawson modification of the Weigert-Sheridan method and counterstained with van Giesons stain. Serial sections were examined from a block of lung in Case 3.

Ebstein's disease

Abstract Four patients with Ebsteins disease are described and an account of the autopsy findings is given in two. Breathlessness on exertion, cyanosis and palpitation were common symptoms. On physical examination there was a praecordial systolic murmur and a well marked triple rhythm at the apex in all four patients. Two patients were dull to percussion to the right of the sternum. Central cyanosis was present at rest in one and during attacks of paroxysmal tachycardia in another. There was electrocardiographic evidence of right auricular hypertrophy in the form of peaked P waves in three and a right bundle branch block pattern in two. On radiologic examination all the patients had an enlarged globular cardiac silhouette mainly due to the abnormal right atrium. Angiocardiography showed a huge right atrium in all cases and early filling of the left atrium in one case subsequently found to have a patent foramen ovale at autopsy. Three of the four patients were investigated by cardiac catheterization and in each case the catheter coiled in a large right atrium. The right atrial pressure was raised in two; only in one was it possible to catheterize the pulmonary artery and in this instance the pressures were normal. At autopsy the abnormalities characteristic of Ebsteins disease were found in both cases. A patent foramen ovale was present in only one of these. The pulmonary vessels were examined histologically in one case and showed changes normal for the age of the patients. There is at present no indication for surgical treatment of this anomaly.

Cor triloculare biatriatum.

Cor triloculare biatriatum is a rare form of congenital heart disease that needs to be considered in the differential diagnosis of the important group of patients with cyanotic congenital heart disease with pulmonary hypertension. The clinical and pathologic features of an example of this disease are reported and discussed in a review of previously reported cases. The small pulmonary blood vessels showed some features of hypertensive pulmonary vascular disease and evidence of an increased bronchial arterial collateral circulation to the lung. An interpretation of these histologic changes relating them to the abnormal pulmonary physiology in cor triloculare biatriatum is suggested.