Right heart catheterization of anomalous pulmonary veins emptying into the right atrium.

Abstract

As the result of the numerous developments in cardiovascular surgery that have taken place during the past decade, the need for a precise diagnosis in a patient with congenital heart disease is obvious. In some patients, operable cardiovascular defects can be diagnosed by relatively simple methods (1, 2). In others, additional studies are necessary in order to make a correct diagnosis. These may include determination of circulation time, angiocardiography, heart catheterization, electrokymography, oximeter studies, unipolar lead electrocardiography, etc. During the course of a study in which we have been evaluating these various diagnostic tests in the diagnosis of congenital heart disease, we have discovered by means of right heart catheterization the anomalous insertion of one or more pulmonary veins into the right atrium. This occurred in three patients clinically suspected of having an interatrial septal defect: Case 1: I. C., a 15-year-old girl, was referred to the University of Minnesota Hospitals by her doctor for evaluation of her heart condition. She was thought to have both acquired and congenital heart disease. When she was three years of age, she was examined because of whooping cough, and the parents were informed at that time that she also had heart disease. At six years of age, she was seen by a cardiologist, who informed the parents that the child had congenital heart disease. She was hospitalized for rheumatic fever for a period of one year at the age of eight, and had a recurrence of that disease when fourteen years of age. At the time of her examination by us, she had minimal cardiac disability. She had no unusual dyspnea, but she complained of occasional bouts of palpitation on exertion. On physical examination the patient appeared well developed and well nourished. The general examination was negative. Examination of the heart revealed a loud, blowing systolic murmur heard best over the pulmonic area but also over the entire precordium. A diastolic murmur was audible over the mitral area. The blood pressure was 120/70 mm. of mercury in the right arm. The clinical impression was that the patient had Lutembacher's syndrome (interatrial septal defect with mitral stenosis). An electrocardiogram revealed right axis deviation. Fluoroscopic examination and roentgenograms of the heart showed a small aorta, a markedly enlarged right ventricle, and an increase in the size of the pulmonary artery, root shadows, and vascular markings. The findings were consistent with an interatrial septal defect. Right heart catheterization was done under local anesthesia. The left median basilic vein was used. A No. 8 ureteral catheter was inserted and advanced into the heart and out into the right pulmonary artery. The oxygen content of the blood in the pulmonary artery and the various chambers, with the pressures, are given in Table I.