Total anomalous drainage of pulmonary veins with venous obstruction . Pulmonary venous obstruction is not unique to the abnormal venous connection below the diaphragm and can occur in the hole where the common vein penetrates or narrows somewhere along its path.
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- 1 Concept and pathological anatomy
- 1 Clinical picture
- 2 Radiological examination
- 3 Electrocardiographic examination
- 4 Diagnosis and differential diagnosis
- 2 Source
Concept and pathology
Total venous connection of the pulmonary veins with venous obstruction is frequent in cases where the common pulmonary vein connects to the umbilicovitelline system, but occasionally it may be in other sites, such as the left innominate vein, the coronary sinus, in the right superior vena cava or directly into the right atrium.
The clinical picture is severe, manifested by cyanosis from the first days of birth and rapidly progressing congestive heart failure. If not diagnosed and treated, children die within the first few days of birth, or weeks. On examination, a soft or absent murmur and no evidence of cardiomegaly on radiological examination. Hepatomegaly always present and peripheral edema at times.
Dyspnea and cyanosis occur in the first days of birth with rapid progression of symptoms, and death can occur in the first days or months.
The heart is normal in size or with moderate growth. Increased vascularity of the venous congestive type and septal lines. The Kerley B lines are dense, straight and are seen at the costofrenic angle and in the lateral view of the chest behind the sternum. The diffuse vascular pattern and reticular appearance.
Electrical signs of right ventricular growth invariably and signs of right atrial growth are not always present.
Diagnosis and differential diagnosis
In the frontal radiological sample of the thorax, the cardiac area is normal and the pulmonary vascularity is venous congestive. Frequently no murmur is heard and when heard it is of low intensity and in the area of the lung. The liver increased in volume, variably.
With the syndrome of hypoplasia of the left cavities due to aortic atresia, which is the most frequent cause of congestive heart failure in the newborn in the first week of life. Echocardiographic study is useful for diagnosis; observed: right ventricular growth, minimal left ventricular cavity and small left atrium, in addition, poor echoes of the mitral valve. In tricuspid atresia, left ventricular growth and QRS axis in the left upper quadrant. In pulmonary valve atresia, decreased pulmonary vascularity.
In transposition of the great vessels, the heart has an ovoid shape, cardiomegaly and increased vascularity and of the left-to-right short-circuit type. With coarctation of the aorta, the pulses in the lower extremities are absent and the blood pressure is higher in the upper extremities than in the lower ones. With hyaline membrane syndrome due to the clinical and radiological symptoms that define the diagnosis.