Téma:  Case Studies

Congenital agenesis of the portal vein as a rare cause of serious pulmonary hypertension – summary of the problematics and a case report

Marcela Tavačová, Monika Kaldarárová, Iveta Šimková

Congenital agenesis of the portal vein (CAPV) as a result of abnormal early embryogenesis is very rare. This condition leads to an abnormal blood flow directly from the portal to systemic circulation via persistent ductus venosus or other congenital portosystemic shunt. Portopulmonary hypertension (PPH) is a relatively isolated complication of portal hypertension with or without associated liver disease. Most articles deal with PPH related to liver cirhosis; cases of PPH due to congenital agenesis of the portal vein are published less frequently. Pathophysiology, clinical presentation, diagnosis and treatment of PPH due to CAPV is to a great extend modified not only by the degree of pulmonary hypertension but also by the severity of liver affection; as well as by the type of portosystemic shunt and other possible congenital anomalies. The management of this complex disease is therefore multidisciplinary, in the hands of experts from several disciplines. A case of a 31-year old male patient with congenital agenesis of the portal vein with portosystemic shunts leading to severe portopulmonary hypertension, is described.

Ročník 2010  Témy časopisu Cardiology for practice 3 / 2010

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MUDr. Peter Dědič
MUDr. Juraj Dúbrava, PhD., FESC
MUDr. Viliam Fridrich, PhD.
doc. MUDr. Ján Kmec, PhD.
MUDr. Gabriela Kaliská, PhD., FESC
MUDr. Pavol Lesný
MUDr. Monika Kaldarárová, PhD.
MUDr. Peter Margitfalvi
prof. MUDr. Daniel Pella, PhD.
prof. MUDr. Iveta Šimková, CSc., FESC
MUDr. Viera Vršanská, CSc.

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Examples of citations:
1. Shaheen NJ, Crosby NA, Bozymski EM, et al. Is there publication bias in the reporting cancer risk in Barrett´ esophagus? Gastroenterology 2000; 119: 333-338.
2. Stenestrand U, Wallentin L. Swedish Register of Cardiac Intensive Care (RIKS-HIA): Early statin treatment following acute myocardial infarction and 1-year survival. JAMA 2001; 285: 430-436.
3. LIPID Study Group. Prevention of cardiovascular events and death with pravastatin in patients with coronary heart disease and a broad range of initial cholesterol levels. N Engl J Med 1998; 339: 1349-1357.
4. Jurkovičová O, Spitzerová H, Cagáň S. Komorové arytmie a náhla srdcová smrť pri akútnom infarkte myokardu. Bratisl Lek Listy 1997; 98: 379-389.
5. Osborne BE. The electrocardiogram of the rat. In: Budden R, Detweiler DK, Zbinden G. The rat electrocardiogram in pharmacology and toxicology. Oxford: Pergamon Press 1981: 15-27.

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In which patients, after overcoming of a thromboembolic cerebral or peripheral event, the least suitable catheter closure is foramen ovale patents:
a. a woman before planned pregnancy
b. paroxysmal fibrillation of the atrium
c. aneurysm of the atrium septum
d. origination of an event after cough

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