JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
DIAGNOSIS: Atrial fibrillation with a ventricular response of 90 beats/minute and right ventricular hypertrophy as indicated by a right-superior QRS complex in the frontal plane (+185°), a qR complex in lead V 1 , S waves in lead V 5 > 0.7 mV (when 0.1 mV = 1 mm), R waves in lead aVR > 0.5 mV, and RV 1 + SV 5 > 1.0 mV. 1 Many congenital cardiac malformations can cause cyanosis, right ventricular hypertrophy, and a systolic murmur, and Fallot’s tetralogy (right ventricular outflow obstruction, ventricular septal defect, right ventricular hypertrophy, and dextroposition of the aorta) tops the list of candidates. As is often the case, an echocardiogram revealed the correct diagnosis. The echo showed pulmonic atresia, the ultimate right ventricular outflow obstruction, as well as the other three criteria for Fallot’s tetralogy. In addition, echo-Doppler revealed a bidirectional shunt through the ventricular septal defect, aortic stenosis, moderately severe aortic regurgitation (thus, the diastolic murmur), and poor left ventricular systolic function. In a necropsy study of 45 patients with Fallot’s tetralogy pulmonic atresia was found in seven with the right ventricular outflow obstruction having been due to infundibular and/or pulmonic valvular stenosis in the other 38. 2 Although aortic valvular abnormalities are considered to be rare in patients with Fallot’s tetralogy, in the study just cited, the aortic valve was morphologically abnormal in 5 of the 45, but in only two was the abnormality hemodynamically significant: a regurgitant congenitally bicuspid valve in one, and a stenotic tricuspid valve in a patient with three episodes of acute rheumatic fever as a child. 2
The patient’s atrial fibrillation was new on this admission. He was severely hypoxic throughout his 23-day hospital stay with an arterial p0 2 ranging from34-44mmHg and arterial O 2 saturations ranging from 51-76% while breathing 40-50% oxygen. He was markedly polycythemia with blood hematocrits ranging from 56-63% and hemoglobins ranging from 18 g/dL to 20 g/dL. His serum creatine (2.1 mg/dL) and blood urea nitrogen (53 mg/dL) were elevated on admission, but returned toward normal with hydration. His serum albumin (2.3 g/dL) was low. Two serum digoxin levels (1.9 and 2.2 ng/mL) were at the upper limit of normal. Serum troponin level (reference < 0.04 ng/mL) was 0.20 on admission. It fell to 0.14 on the 19 th hospital day and then rose to 0.46 on the 22 nd hospital day. On those same 3 days the serum myoglobin level (reference 14.3 - 65.8 ng/mL) was 24.0, 26.6, and 195.0 ng/mL, respectively. Other serum laboratory values were normal or nearly so. The cause of the patient’s diarrhea was never determined, and it continued. The serum potassium (reference 3.6 mEq/L to 5.0 mEq/L) was 4.6 on admission and gradually fell to 3.8 mEq/L. On the 23 rd hospital day the patient had multiple episodes of polymorphic ventricular tachycardia (Figure 2), followed by a slow junctional rhythm that terminated in asystole and death. Despite his multiple problems, at age 42 he had outlived nearly all patients with Fallot’s tetralogy, who rarely make it to 30 years of age without operation. 3
Figure 2: Lead II (above) and lead V1 (below) telemetry rhythm strips showing polymorphic ventricular tachycardia. Soon after this recording the patient died in asystole.
J La State Med Soc VOL 170 JANUARY/FEBRUARY 2018 29
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