Case Descriptions
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Case Descriptions Case 1 (A 7645, 30 years, 15 July 47)* Clinical History This 30-year-old patient was admitted to the hospital for the second time with convulsions,S days prior to the date of expiration (second admission). This patient turned blue when crying as a baby. The mother was told that the child had heart disease when the child was 6 years of age. In 1933, at the age of 16 years, the patient had severe grippe with marked cyanosis, swollen and painful joints. Diagnosis of rheumatic fever was made. Following this the patient would turn blue on the slightest exertion. In May, 1947, she had a generalized convulsive seizure followed by several similar seizures. One observer reported that the twitching always occurred on the right side. She was admitted to the hospital on June 3, 1947. Diag nosis of ventricular septal defect, pulmonary stenosis, dextroposition of the aorta, hypertrophy of the right ventricle, and convulsions due to cerebral thrombosis was established. A Blalock procedure (left subclavian artery to left pulmonary artery) was performed. On examination the patient was found to be having a convulsive seizure that started in the right hand and spread to the right and then the left leg, then tonic movements of the head and neck lasting 60 sec. Unconsciousness lasted 15 sec. Cyanosis of the fingers and toes was noted as was clubbing of same. Speech was slurred; fundi were normal. Right facial weakness was noted. There was abnormal rhythm and rate of the heart and a loud systolic murmur. Blood pressure (BP) was 140/90. Pulse (P) was 100; respiration (R) was 26. Absence of left radial pulse was noted. Neurological exam revealed active reflexes on both sides with a positive Babinski on the right. Treatment with infusions was begun. Diagnosis of cerebral thrombosis, probably of one of the middle cerebral branches, was made. Tempera ture (T) became elevated to 101 0 F on the 2nd day only. The patient expired on the 5th hospital day. Lab Data (11 Ju147)t Blood: RBC Hgb WBC N. Seg Seg Ly E. M. Hct 5.8 19 11,800 2 72 15 4 53 CO. of 49.5 vol. percent • The items in parentheses refer to the patient file identification num ber, age at death, and date of death. t RBC, red blood cell count; WBC, white blood cell count; Hgb, hemoglobin g%; N. Seg, non segmented; Seg, segmented; Ly. lymphocytes; E, eosinophils; M, monocytes; Hct, hematocrit. 149 150 Case Descriptions Autopsy Diagnosis Congenital heart disease with pulmonary stenosis; dextroposition of aorta; interventricular septal defect; hypertrophy of right ventricle; patent foramen ova Ie; patent ductus arteriosus; healed aortic and pulmonic valvular endocarditis with stenosis; chronic abscess of brain (left frontoparietal area); multiple pulmonary and pleural scars. Case 2 (A 7703, 3 years, 9 Sep 47) Clinical History This 3-year-old white male child was cyanotic upon exertion from the age of 3 months. His gain in weight and development were slow; his activities voluntarily restricted. When he was admitted to the New Haven Hospital at the age of 2 V2 years, the diagnosis of tetralogy of Fallot was made and an end-to-side anastomosis was established between the right subclavian and pulmonary arteries. Preoperatively, the maximum observed systemic arterial O2 saturation at rest was 38%, which fell with moderate activity of the child to only 27%. Postoperatively the level with moderate activity was 79%. The child was greatly improved after the operation but expired 4 months later during the difficult surgical removal of a large Wilms tumor of the left kidney Autopsy Diagnosis Tetralogy of Fallot; surgical anastomosis of right subclavian artery to right pulmonary artery, old; abdominal incisions, recent, and absence of left kidney (removed for a Wilms tumor); hemoperitoneum. Case3 (A7987, II months, 8 May 48) Clinical History This II-month-old white female child had been noted to be cyanotic upon exer tion at 6 weeks of age and from the age of \0 weeks was at least slightly cyanotic at all times. The diagnosis of tetralogy of Fallot was made at the age of II weeks. At that time an arterial O 2 saturation determined when she was crying and exhausted was 23%. At the age of 7 months she developed the signs of a cerebral arterial thrombosis. Because of this unfavorable incident, a Blalock-Taussig operation was performed I month later, anastomosing the left subclavian and left pulmonary arteries. The patient's color remained pink for 24 hr postoperatively, but then the murmur of the surgical shunt disappeared and cyanosis recurred to the same degree as before. It was apparent that in spite of intravenous heparin therapy the anas tomosis had become occluded. Three months later, at the age of II months, another operation was performed, this time anastomosing the right subclavian and the right pulmonary artery. After 24 hr of an encouraging postoperative course, she suddenly became dyspneic and cyanotic and expired a few minutes later. Case Descriptions 151 Autopsy Diagnosis Tetralogy of Fallot; old operative anastomosis of left subclavian artery with thrombosis of anastomosing vessels; recent operative anastomosis of right subcla vian artery to pulmonary artery with fresh thrombus at site of anastomosis; focal atelectasis of lungs; old and recent encephalomalacia. Case 4 (A8026, 7 years, 26 Jun 48) Clinical History This 7-year-old white girl had been cyanotic upon exertion since infancy. Although her exercise tolerance was low, her general development was quite normal. When she was admitted to the hospital at the age of 7 years, the physical and fluoro scopic findings indicated the presence of tetralogy of Fallot, as well as a right aortic arch. The right ventricular pressure measured by cardiac catheterization was 60 cm H20. The systemic arterial O2 saturation while the patient breathed room air at rest was 63.5%. While she breathed pure O2 it rose to 77.5%. A Blalock-Taussig opera tion was begun, but during the initial dissection around the left subclavian artery the patient suddenly expired with visible ventricular fibrillation. Autopsy Diagnosis Tetralogy of Fallot; patent foramen ovale; right-sided aortic arch; recent inci sion left anterior chest; clubbing of fingers and toes; mucosal hemorrhage of stomach. Case 5 (A8031, I V2 years, 30 Jun 48) Clinical History This 18-month-old white female infant was noted to have a systolic murmur at birth and to be cyanotic with exertion at the age of 4 months. The diagnosis of tetralogy of Fallot was made, but, since the child's general condition seemed satis factory, operation was deferred until a more opportune age. Cyanosis became more marked during the following year, with occasional episodes of paroxysmal dyspnea. At the age of II months, an arterial O2 saturation while the child was crying and totally exhausted was only 12.3%. There was some question as to whether venous blood might not have been accidentally withdrawn for the analysis, but it was esti mated that, even if this were true, the arterial O2 saturation could not have been over 35%. At the age of 18 months, a Blalock-Taussig operation was performed, ana stomosing the left subclavian and left pulmonary artery. The postoperative course was uneventful for the first 12 hr, but the respiratory distress became evident and continued until death 12 hr later. Heparin had been administered by continuous intravenous drip during this 24-hr period, but the blood clotting levels were difficult to regulate and were rarely satisfactorily low. 152 Case Descriptions Autopsy Diagnosis Tetralogy of Fallot; interauricular septal defect; thrombus in left subclavian and left pulmonary artery at site of anastomosis; interstitial emphysema of left lung. Case 6 (A8119, 46 years. 24 Sep 48) Clinical History This 46-year-old white male was dead on arrival at Grace-New Haven Com munity Hospital (GNHCH) on September 24, 1948. His first admission here dated back to 1938 when he was 36 years old. He was given a preemployment physical examination with a history of heart trouble since birth with no symptoms. There was a question of cyanosis after birth. He had had active TB from the ages of 7-11 years. While at Gaylord Farms the diagnosis of congenital heart disease was made. Physical examination on admission here in 1938 revealed a BP of 245/120. There was marked cardiomegaly. A loud, rough systolic murmur was heard over the entire chest and best in the apical region. There was definite pulsation of the inter costal arteries bilaterally. The arm blood pressure on the left was 255/125, while the leg blood pressure was O. The dorsalis pedis and posterior tibial pulses were absent. X rays revealed notching of the ribs and a diagnosis of coarctation of the aorta was made. In February, 1948, the patient was admitted after the onset of ankle edema and cyanosis and bloody stools over a 2-week period. Physical examination (PE) then showed moderate cyanosis of the hands and feet. Marked edema of the ankles and legs was present. No peripheral pulses were obtained in the lower extremities. The heart was enlarged with a Grade II systolic harsh murmur transmitted over the pre cordium. Dullness was present at both lung bases. The liver edge was 2 cm below the costal margin. Electrocardiogram (EKG) showed left-axis shift with the suggestion of anterior wall infarction. X rays revealed rib notching with cardiomegaly. Gastrointestinal series suggested a duodenal ulcer. The patient was digitalized but his feet became progressively more gangrenous. Supracondylar amputation of the right leg and of the left toes was carried out. The patient was discharged as improved. Autopsy Diagnosis Coarctation of aorta; generalized arteriosclerosis with particular involvement of coronary and cerebral arteries; incomplete occlusion of anterior descending branch of left coronary artery; infarct and scar of apex of heart; mural thrombosis in wall of left ventricle; hypertrophy and dilatation of left ventricle; absence of right leg and left toes; recanaIized thrombus in both iliac veins, fresh and organized infarcts in right middle and lower lobe; pleural adhesions, right; right hydrothorax.