Twenty-five consecutive patients with obliterative pulmonary hypertension were studied. Primary pulmonary hypertension (17 patients) or chronic thromboembolic pulmonary hypertension (eight patients) was diagnosed by pulmonary angiography or autopsy. Clinical symptoms, physical findings, chest roentgenograms, electrocardiograms, and pulmonary function studies did not differentiate the patients with primary pulmonary hypertension (PPH) from those with chronic thromboembolic pulmonary hypertension (TPH). All eight patients with TPH had a lung scan interpreted as high probability for pulmonary emboli while all 17 patients with PPH had a lung scan interpreted as normal or low probability for emboli. While there was close clinical similarity between patients with PPH and TPH, the presence of a normal or low probability lung scan excluded the diagnosis of TPH.
We report three siblings who presented with a clinical picture of persistent pulmonary hypertension of newborn and died between 4 and 15 days of age. Pulmonary artery pressure in all was above systemic values, with a right to left shunt via either the foramen ovale or ductus arteriosus, or both. Histology of the pulmonary vascular bed showed extension of muscle into small arteries which are normally non-muscular.
Over a period of five years 12 patients underwent electrophysiological studies for the investigation of recurrent tachycardias which showed ventricular pre-excitation. Nine patients had a type B pattern and two a type A. One patient had episodes of both types. Dual atrioventricular nodal pathways were found in six of seven patients with atrioventricular nodal re-entrant tachycardia mechanisms. Single direct atrioventricular accessory pathways were present in four patients, single nodoventricular pathways in five, and multiple pathways in three. Twenty one tachycardias were induced, of which 13 showed ventricular pre-excitation. Five patients had nodoventricular pathway conduction during atrioventricular nodal tachycardia and one during atrioventricular re-entrant tachycardia. Only three patients had simple antidromic tachycardia and one additional atrioventricular nodal tachycardia with bystander atrioventricular accessory conduction. Three patients had three different tachycardias, three had two types, and six had one type. Thus junctional tachycardias showing ventricular pre-excitation are often associated with multiple mechanisms and complex anatomical and functional substrates. An accessory pathway was an essential component in only six of 13 tachycardias showing ventricular pre-excitation. Determination of the tachycardia mechanism requires detailed study and analysis.,BACKGROUND: In patients with primary pulmonary hypertension who respond to vasodilators acutely, survival can be improved by the long term use of calcium channel blockers. However, testing for such a response with calcium channel blockers or prostacyclin (PGI2) may cause hypotension and adversely affect gas exchange. Nitric oxide (NO), which does not have these effects, could be a better test agent. METHODS: NO (10, 20, and 40 ppm for 15 minutes), PGI2 (1>10 ng/kg/min), and oral nifedipine (10 mg, then 20 mg/h) were administered sequentially to 10 patients after determination of the 24 hour spontaneous variability of their pulmonary and systemic mean arterial pressures. Patients were considered responders if the mean pulmonary artery pressure or pulmonary vascular resistance decreased by 20% or more. RESULTS: Six patients (60%) responded to all three agents, and three to none of the agents. One patient responded to PGI2 only. In those who responded to vasodilators, NO had no major effect on gas exchange or systemic haemodynamics, while PGI2 and nifedipine both induced systemic hypotension (mean (SD) systemic arterial pressure 72 (14) versus 89 (19) mm Hg with PGI2 and 72 (15) versus 86 (17) mm Hg with nifedipine, p < 0.05) and hypoxaemia (PaO2 8.7 (1.4) versus 10.8 (1.0) kPa with PGI2 and 8.6 (1.4) versus 10.2 (1.5) kPa with nifedipine, p < 0.05) and increased venous admixture (28 (9) versus 14 (4)% with PGI2 and 22 (9) versus 13 (5)% with nifedipine, p < 0.05). CONCLUSIONS: NO inhalation can accurately predict a vasodilator response to nifedipine in patients with severe pulmonary hypertension without adverse effects on systemic haemodynamics and gas exchange. This absence of side effects may make it a more appropriate agent for testing the vasodilator response.
Blood flow patterns were analysed at nine points in the pulmonary area using the pulsed Doppler technique combined with cross-sectional echocardiography in 53 patients with heart disease and 10 healthy subjects. In subjects with a normal pulmonary artery pressure the blood flow pattern in systole showed a gradual acceleration and deceleration with a rounded summit in mid systole, designated the round type. In patients with pulmonary hypertension it showed a rapid acceleration and early deceleration with a sharp peak in early systole, designated the triangular type. The acceleration time index, defined as the ratio of the time interval from the beginning to the peak of ejection to the ejection time, showed a significant inverse correlation with mean pulmonary artery pressure. In pulmonary hypertension a prominent reverse flow occurred in the right posterior part of the pulmonary trunk during mid-systole and early diastole, indicating the presence of a vortex. Similar flow patterns were also seen in patients with idiopathic pulmonary artery dilatation. The factors responsible for the triangular type were principally the reduced capacitance and increased impedance of the pulmonary vascular tree. Those responsible for the reverse flow were the curved path of the blood flow and dilatation of the pulmonary artery.,BACKGROUND: A new portable spirometer, the Spirophone, has been developed that records a subject's blow and can then transmit all the data by telephone to a receiving centre for analysis and comment. Tests of this device were undertaken to determine its accuracy and reliability. METHODS: The performance of the Spirophone was tested using computer generated wave forms, by delivering blows from calibrated syringes at different flows, and by comparing subjects' blows with those recorded with a commercial spirometer. RESULTS: Using computer generated wave forms all lung function indices were accurate to within 1% and blows delivered from calibrating syringes were accurate to within 5%. When subjects performed repeated forced vital capacity (FVC) manoeuvres there were no significant differences between lung function indices recorded with the Spirophone and with a commercial spirometer. With the Spirophone and commercial spirometer in series the FVC and forced expiratory volume in one second (FEV1) were within 5% of each other in nine out of 10 healthy subjects. CONCLUSION: The Spirophone recorded maximal forced expiratory manoeuvres with acceptable accuracy, reliability, and reproducibility, and this system offers the ability to monitor a patient's lung function at a centre remote from the patient.
Combined heart and lung transplantation was carried out in 17 patients at Stanford University between March, 1981, and December, 1983. The recipients were between 22 and 45 years old. All patients had end-stage pulmonary hypertension; 10 had Eisenmenger's syndrome and the remaining 7, primary pulmonary hypertension. Five patients died within the first few postoperative weeks. The remainder are well between four weeks and 33 months from operation. The immunosuppressive protocol has consisted of cyclosporine with an initial course of rabbit antithymocyte globulin. Azathioprine also was given for the first two weeks and then was replaced with prednisone. Rejection, as diagnosed by cardiac biopsy, was treated with high doses of methylprednisolone. Modifications of technique that have developed include the removal of the recipient heart and lungs separately, and preservation of the lungs with a modified Collins' solution instead of a cardioplegic solution. Rejection occurred in 6 of the 12 survivors. Infections developed in 9 patients, but only one resulted in a fatal outcome ( ). Thus, the results of clinical heart-lung transplantation have been considerably superior to clinical efforts in lung transplantation. It is suggested that the combined operation is preferable for the following reasons: (1) all diseased tissue is removed, thus eliminating recurrent infection and ventilation/perfusion disparity; (2) transplantation of the entire heart-lung block preserves coronary–bronchial vascular anastomoses and makes airway dehiscence less likely; and (3) to date, diagnosis of rejection by cardiac biopsy has appeared to be a satisfactory method of diagnosing and treating pulmonary rejection. Cardiopulmonary transplantation represents a viable therapeutic approach for patients with end-stage pulmonary hypertension with or without associated congenital heart disease.
A patient with a circulating lupus anticoagulant in the absence of systemic lupus erythematosus developed recurrent deep venous thromboses and pulmonary emboli. Pulmonary emboli recurred despite prolonged oral anticoagulant therapy and resulted in fatal pulmonary arterial hypertension. Extended anticoagulant therapy alone may not prevent recurrent thromboembolism in patients with a lupus anticoagulant. Pulmonary thromboembolism may be an important factor in the pathogenesis of pulmonary hypertension in patients with a lupus anticoagulant.