Developmental disorders

III./4.3.: Symptomatology

The clinical signs of complex heart defects such as DORV may appear based on the absence or presence of pulmonary stenosis and localization of VSD DORV constitutes the category of conotruncal disorders from Fallot IV to great vessel transposition (TGA).

III./4.3.1.: DORV with pulmonary stenosis

Signs may mimic signs of Fallot IV with cyanosis (decreased pulmonary circulation). Main features: neonatal cyanosis, arterial desaturation, dyspnea, feeding difficulties, growth and developmental retardation. Auscultation provides loud ejection murmur of pulmonary stenosis on the left side of the sternum in the II. Intercostal space, which directed toward the lungs. In case of sever pulmonary stenosis, the neonate’s circulation mat depend on the patent duct, thus its closure may result in circulatory arrest.

III./4.3.2.: DORV without pulmonary stenosis

Both great vessels are originated from the right ventricle, the VSD is subaortic. Clinical signs are similar to the non-complicated VSD, in which left to right shunting is significant. Mild systemic desaturation may be present at early onset. Major left to right shunting may present with signs and symptoms of pulmonary hypertension and congestive cardiovascular failure. Respiratory disorders are frequent. Cyanosis is moderate.

III./4.3.3.: Taussig-Bing-anomaly

VSD may appear subpulmonal, or underneath both semilunar valves in this type of DORV; great vessel position is side by side. Because of the direction of intracardial circulation clinical signs may mimic great vessel transposition with VSD and subsequent pulmonary hypertension. Sever cyanosis is observed at birth, which accompanied by circulatory failure. The risk of the development of early pulmonary vascular disease is high. Auscultation provides systolic murmur and ejection click most often and loud II. sound above the auscultation point of the pulmonary valve.

III./4.3.4.: Diseases most often associated with DORV

• – Mitral atresia and hypoplastic left ventricle

• – Tricuspid stenosis

• – Ebstein-anomaly

• – Complete form of AVSD

• – Aortic coarctation

• – Aortic arch interruption

• – Heterotaxia

III./4.3.5.: DORV associated syndromes

• - trisomy13

• - trisomy18