JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
DIAGNOSIS: Sinus rhythm at a rate of 76 beats/minute; a slightly long P-R internal (0.22 seconds); a right superior QRS axis (-153°); right atrial enlargement (P wave in lead V2 > 0.15 mV)1; right ventricular hypertrophy (qR pattern in lead V1 suggested right ventricular systolic pressure ≥ left ventricular systolic pressure). 2 What has caused this cyanosis from birth and striking right ventricular hypertrophy? The absence of a murmur rules out tetralogy of Fallot and other forms of right ventricular outflow obstruction with a right-to-left shunt. A precordium devoid of palpable or auscultatory abnormality in a cyanotic patient has always been one of the hallmarks of the Eisenmenger syndrome. Echocardiography on the current admission revealed a large ventricular septal defect with a bidirectional shunt and no pressure gradient across the defect (thus, no murmur). Records from the hospital where the operation was performed revealed that the patient had a double outlet right ventricle with an anterior aorta. Because the large left-to-right shunt was responsible for the failure to thrive, banding of the pulmonary trunk was attempted. That was when ventricular fibrillation occurred. The bandwas removed, and the right pulmonary artery was banded. The arterial vasculature of the left lung continued to be damaged by high pressure and high flow. Meanwhile the right pulmonary artery thrombosed, leaving no significant flow to the right lung. The patient clearly has never been a candidate for complete repair of his congenital cardiac defect. Heart-lung transplantation has not been an option because of his drug and alcohol history. Intermittent phlebotomy keeping his hematocrit and hemoglobin levels at approximately 53% and 17 gm/dL, respectively, have relieved his headaches, and oral iron has prevented iron deficiency.
REFERENCES
1. Surawicz B, Knilans TK. Chou’s Electrocardiography in Clinical Practice: Adult and Pediatric, 5th edition. Philadelphia: W.B. Saunders;2001:31. 2. Marriott HJL. Pearls and Pitfalls in Electrocardiography: Pithy, Practical Pointers. Philadelphia: Lea & Febiger;1990:56.
Dr. Glancy is an emeritus professor in the Department of Medicine, LSU Health Sciences Center, New Orleans, Louisiana.
J La State Med Soc VOL 169 NOVEMBER/DECEMBER 2017 177
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