Abstract

 
 
 Congenital heart disease (CHD) is formed during intrauterine period but, as a rule, they are not hereditary. Hemophilia A belongs to the group of hereditary coagulopathies stipulated by deficiency of coagulation factor VIII and characterized by bleeding of hematoma like type. Combination of these two diseases is rather rare that requires cardiosurgeons’ apprehensive attitude.We present our experience in surgical treatment of a boy of 8 months old with Tetralogy of Fallot. Hemophilia A was found in preoperative examination. He was successfully operated with cardiac pulmonary bypass on the background of factor VII injection.Surgical treatment was successful without significant bleeding. Postoperative period was uneventful. For successful treatment of CHD with coagulation failure is very important timely diagnosis, that can be difficult due to the lack of specific symptoms in children.Surgical treatment is the method of choice in TOF. Timely and complete diagnosis of comorbidities allows to make it safe even in case of Hemophilia A.
 
 
Highlights
Congenital heart disease (CHD) is formed during intrauterine period but, as a rule, they are not hereditary
Tetralogy of Fallot (TOF) is the complex anatomical anomaly of the heart stipulated by undevelopment of the right ventricle infundibulum
TOF may be combined with other cardiac malformations: right sided arch of the aorta (20-25%), vascular ring, secondary atrial septal defect (ASD), patent ductus ateriosus (PDA), patent common atrioventricular canal, persistent left superior vena cava, and partial drainage of pulmonary veins
Summary
Congenital heart disease (CHD) is formed during intrauterine period but, as a rule, they are not hereditary. Genes stipulating development of hemophilia are X-linked; due to it the disease runs in the family by recessive inheritance through female lineage. Combination of these two diseases is rather rare that requires cardiosurgeons’ apprehensive attitude [1]. There are four components of this malformation in the typical case: stenosis of pulmonary artery, non-restrictive ventricular septum defect (VSD), dextraposition of the aorta and hypertrophy of the right ventricle. TOF may be combined with other cardiac malformations: right sided arch of the aorta (20-25%), vascular ring, secondary atrial septal defect (ASD), patent ductus ateriosus (PDA), patent common atrioventricular canal, persistent left superior vena cava, and partial drainage of pulmonary veins. To review our experience of the surgical treatment of child with TOF and Hemophilia A, the importance of timely and detailed diagnostics for the optimization surgical and therapeutic approach
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