Double Inlet Left Ventricle with Eisenmenger Syndrome in an Adult – A Case Report
Keywords:Heart Defects, Congenital, Transposition of Great Vessels, Polycthemia, Eisenmenger syndrome; Pruritis
Background: Patient diagnosed with double inlet left ventricle (prevalent in 5 – 10 in 100,000 newborns) complicated with Eisenmenger syndrome had a median survival age of 14 years without corrective surgery. Congenital heart disease such as this is usually treated by multiple surgeries during early childhood. A surgically uncorrected case in adults is not of common occurrence. Further, generalized itching after coming in contact with water (aquagenic pruritis) presented an interesting conundrum to treat.
The Case: A 29-year-old patient in India presented at a primary health care center with a history of difficulty breathing and discoloration of extremities since birth. He also gave a history of itching which commonly occurred after taking bath, hemoptysis and history of turning blue in color after birth. Patient had received no treatment besides regular phlebotomies. On examination, there was grade IV clubbing and conjunctival congestion. Cardiovascular examination revealed an enlarged heart, heaving apex beat and a pan-systolic murmur. A provisional diagnosis of a congenital cyanotic heart disease was made. Investigations revealed hemoglobin of 16.8g/dl. X–ray and electrocardiogram showed hypertrophy of the ventricles. An echocardiogram showed double inlet left ventricle with L-malposed vessels but without pulmonary stenosis. A final diagnosis of congenital heart disease; double inlet left ventricle, L-malposed vessels without pulmonary stenosis, Eisenmenger Syndrome and absolute erythrocytosis was made. Patient was advised for further management with a cardiologist in a tertiary center but the patient did not follow up.
Conclusion: Unlike in high-income countries where most congenital heart diseases are detected and dealt with...
2. Koito H, Ohkubo N, Suzuki J, Iwasaka T, Inada M. Prolonged survival in a patient with a single ventricle without pulmonary stenosis. Chest 1994; 106: 971-2. (Review)
3. Baldwin HS, Dees E. Embryology and physiology of the car¬diovascular system. In: Gleason CA, Devaskar S, eds. Avery's Diseases of the Newborn. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 50.
4. Franklin RC, Spiegelhalter DJ, Anderson RH, Macartney FJ, Rossi Filho RI, Douglas JM et al. Double-inlet ventricle presen¬ting in infancy: I: Survival without definitive repair. J Thorac Cardiovasc Surg. 1991;101: 767–776.
5. Moodie DS, Ritter DG, Tajik AJ, O'Fallon WM. Long-term fo¬llow-up in the unoperated univentricular heart.
Am J Cardiol 1984; 53: 1124-8.
6. Jensen AS, Iversen K, Vejlstrup NG, Hansen PB, Søndergaard L (April 2009). "[Eisenmenger syndrome]". Ugeskrift for Laeger (in Danish) 171 (15): 1270–5.
7. Weigel TJ, Driscoll DJ, Michels VV. Occurrence of congenital heart defects in siblings of patients with univentricular heart and tricuspid atresia. Am J Cardiol. 1989; 64: 768–771.
8. Burn J, Brennan P, Little J, Holloway S, Coffey R, Somerville J, Dennis NR, Allan L, Arnold R, Deanfield JE, Godman M, Houston A, Keeton B, Oakley C, Scott O, Silove E, Wilkinson J, Pembrey M, Hunter AS. Recurrence risks in offspring of adults with major heart defects: results from first cohort of British collaborative study. Lancet. 1998; 351: 311–316.
9. Nelson DP, Schwartz SM, Chang AC. Neonatal physiology of the functionally univentricular heart. Cardiol Young. 2004; 14 (Suppl 1): 52–60.
10. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J. Sep 27 1958;2(5099):755-62.
11. Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med. May 1 1998;128(9):745-55
12. Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation. Feb 27 2007;115(8):1039-50.
13. Mohan H. Textbook of pathology. 6th ed. New Delhi: Jaypee Brothers Medical Pub; 2010:Chap 16, pg 421.
14. Abdel Naser MB, Gollnick H, Orfanos CE. Aquagenic pruritus as a presenting symptom of polycythemia vera. Dermatology. 1993;187:130–3.
15. Moodie DS, Ritter DG, Tajik AJ, O’Fallon WM. Long-term follow-up in the unoperated univentricular heart. Am J Car¬diol.1984; 53: 1124–1128
16. Diller GP, Kempny A, Inuzuka R, Radke R, Wort SJ, Baumgart¬ner H et al. Survival prospects of treatment naïve patients with Eisenmenger: a systematic review of the literature and report of own experience. Heart. Sep 2014;100(17):1366-72.
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