A 5-year-old boy with cyanotic heart disease and weak pulses in the left arm is described. Cardiac catheterization and cineangiography confirmed the diagnosis of tetralogy of Fallot and right aortic arch with isolation of the left subclavian artery. In addition to the aortogram and right ventricular cineangiography, pulmonary angiography was performed, demonstrating that blood did not reach the left subclavian artery through a left ductus arteriosus.
A patient with papillary muscle disease caused by myocardial infarction was studied before and after injection ofphenylephrine. The pulmonary wedge pressure was normal at rest. However, pressures and murmur changes, occurring spontaneously and after injection ofphenylephrine, suggested that intermittent severe mitral regurgitation contributed significantly to the recurrent episodes of acute left heartfailure presented by this patient. Case report A 53-year-old woman was admitted with a 6-month history of recurrent pulmonary oedema associated with chest pain. Except for mild diabetes, she had been previously healthy. Between the episodes of acute left heart failure, she was feeling relatively well, complaining only of atypical chest pain and of mild dyspnoea on effort. Clinical examination on admission showed a woman in no distress. Her blood pressure was IIO/70 mmHg (14.6/9.3 kPa) and the pulse was regular, 70/minute.The jugular pulse and pressure were normal, the edge of the liver was palpated 3 cm under the costal margin, and no peripheral oedema was present. The lungs were clear. The left ventricle was enlarged on palpation and a prominent lower left sternal impulse was felt. The first heart sound was reduced in intensity and the pulmonary closure sound was accentuated. A grade 3/6 pansystolic murmur was heard at the apical area, conducted to the axilla, and along the left sternal border, followed by a third heart sound and a short mid-diastolic rumble. The murmur varied only slightly from day to day. The chest x-ray showed moderate enlargement of the left ventricle and left atrium. There were signs of pulmonary venous congestion and of pulmonary arterial hypertension.Acute subvalvar mitral regurgitation was considered to be the most likely diagnosis at this stage. Right heart catheterization was performed under local anaesthesia. Pethidine 50 mg and promethazine hydrochloride 50 mg were given intramuscularly i hour before catheterization. No shunts were detected. The pulmonary artery pressure was 56/24 mmHg (7.4/ 3.2 kPa) at the beginning ofthe procedure. While attempting to record a reliable pulmonary wedge pressure, the main pulmonary artery pressure was observed to vary spontaneously, finally dropping to i8/8 mmHg (2.4/ 1.1 kPa) about 30 minutes after the first measurement was obtained. At that time, the mean pulmonary wedge pressure was 9 mmHg (r.2 kPa), with a v wave of 14 mmHg (I.9 kPa). The systemic blood pressure as measured by sphygmomanometer was unchanged at 110/70 mmHg (14.6/9.3 kPa); the heart rate was 120/ minute. The murmur was barely audible.Phenylephrine, i mg diluted in IO ml of normal saline, was injected intravenously. Within i minute, the blood pressure rose to I50/90 mmHg (I9.9/II.9 kPa) and the heart rate dropped to ioo/minute. A giant v wave appeared on the pulmonary wedge tracing (Fig. i) while the pulmonary artery pressure rose to 84/30 mmHg (II.2/3.9 kPa) (Table). The patient complained of chest pain and of increasing dyspnoea. The murmur became much louder. These changes persisted...
A 22-year-old woman with a right atrial myxoma prolapsing to the right ventricle is described. The haemodynanmc findings were similar to those of cases of prolapsing myxoma of the left atrium; a notching on the ascending limb of the right ventricular pressure curve, and an initial negative, irregular deflection on the pulmonary artery pressure curve with a pronounced rise in the mean right atrial pressure (18 mmHg) were found. On deep inspiration there was a significant deepening of the y descent from 12 mmHg to 2 mmHg, indicating a changing, dynanic obstruction of the right ventricle inflow tract. These haemodynamic features can be helpful in the diagnosis ofprolapsing right atrial myxoma.Myxomata of the atria are uncommon, especially in the right atrium (Sannerstedt et al., 1962;Morrissey et al., 1963). Recently, Sung et al. (1975) differentiated between two types of left atrial myxoma: type I, in which the tumour prolapses during diastole into the left ventricle, and type II, which is non-prolapsing. Sung described a notch in the upstroke of the left ventricular pressure curve and a rapid y descent in the pulmonary arterial wedge pressure curve as characteristic of the prolapsing type.The purpose of this report is to present a case of a right atrial myxoma, prolapsing during diastole into the right ventricle, producing haemodynamic features similar to those described by Sung for the prolapsing left atrial myxoma. Case reportA 22-year-old single woman of Arabian origin had been completely well until 6 months before her admission, when she began complaining of palpitation, frequent cough, and haemoptysis; one month before admission her complaints became worse with pain in the right posterior thorax, frequent nausea and vomiting, and a loss of 7 kg in weight. On admission she had a temperature of 37 9°C; there was no cyanosis, clubbing, or dyspnoea; her blood pressure was 120/80 mmHg, pulse rate 110/ min, with regular rhythm. There was pronounced jugular venous engorgement with prominent a and v waves and a negative Kussmaul sign. Auscultation of the lungs revealed poor air entry into the right lung base and a pleural friction rub at the left base. The apex beat was palpated in the left fifth interspace in the midclavicular line; no right ventricular heave was noted. A diastolic thrill at the lower left sternal border was palpable; the first heart sound was prolonged and accentuated. The second sound was normal; a grade 4/6 mid-diastolic presystolic murmur extending into the first sound and early systole was heard at the lower left sternal border. The character of the murmur was similar to a pericardial friction rub; the intensity of the murmur was unaffected by the position of the patient but increased slightly on deep inspiration. The liver was palpable 5 cm below the costal margin and tender; the spleen was not palpable. There was mild pitting oedema in both legs.The electrocardiogram showed sinus rhythm with a rate of I10/min, tall P waves in leads II, III, and aVF. The QRS axis was + 1200, and there were n...
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