Other Congenital Cyanotic Heart Diseases
Complex Pulmonary Atresia
This is characterized by total discontinuity between the right ventricle and the pulmonary artery. The obstruction may take different anatomical forms. They present with cyanosis and reduced pulmonary flow early in infancy. Two types are recognized depending upon the presence or absence of VSD. Pulmonary atresia with VSD is essentially like the tetralogy of fallot, but the RVOT obstruction is complete. Pulmonary atresia with intact ventricular septum has been divided into two types on the basis of right ventricular (RV) size. In majority of cases, the RV is of diminutive size and in a small percentage RV is of normal size. Blood flows into the pulmonary artery from the aorta through the ductus if it is patent, otherwise, through the bronchopulmonary collaterals. They usually present with progressive neonatal cyanosis, respiratory distress, and early death.
Tricuspid atresia is a complex of several separate anatomic abnormalities. These are:
- Atresia of the tricuspid valve
- Defect in the inter-atrial septum
- Hypoplasia of the sinus portion of right ventricule and’
- Usually a ventricular septal defect.
The great vessels may be normally connected or transposed. The tricuspid valve is atretic and its location is marked by a dimple. The major clinical finding is moderate generalized cyanosis, often present from the first day of life. In cases with large pulmonary blood flow, the left atrium and left ventricle will be dilated and infants present with congestive heart failure.
Transposition Of Great Vessels
Complete Trasnposition Of Great Vein
Complete transposition of great vessels is the most common cardiac anomaly producing cyanosis in the newborn. The venous connections of the heart are normal, the ventricles occupy normal position, but the right ventricule is connected to aorta which is anterios and the left ventricule is connected to pulmonary artery which is posterior. The two circulations are parallel, independent of each other. Right ventricle pumps blood into the aorta and this blood is returned by the systemic veins and the vena cavae into the right atrium. Left ventricle pumps blood into the pulmonary artery and this blood is returned by the pulmonary veins into the left atrium. Life is sustained by the mixing of blood returning from the pulmonary and the systemic veins through VSD, ASD or PDA. Most often it presents with cyanosis and increased pulmonary blood flow and such cases lead on to congestive heart failure.
Mitral Valve Called Out
Mitral Valve Prolapse Syndrome
Also known as Midsystolic click syndrome, Floppy valve syndrome or Barlow’s Syndrome. This anomaly is seen in 3-5% of apparently healthy population. The mitral valve cusps are redundant and they prolapsed into the left atrium during systole. The collagen of the valve is abnormal. Associated rated abnormalities include rupture of the chordate tendinae and ventricular dysfunction.
Most cases are asymptomatic and the anomaly may be accidentally detected. In symptomatic cases, the complaints include chest pain, palpitation or ventricular ectopics. Auscultation reveals midsystolic click and late systolic murmur, better heard when the patient stands. In severe cases mitral incompetence may be detectable. Complications are rare. These include infective endocarditis and thromboembolic episodes. The course is benign in the vast majority of cases.
This ailment is confimed by echocardiography and angiocardiography. Sedatives and betablockers (Propanolol) control the tachycardia and ectopics. Prophylaxis for infective endocarditis should be instituted.
© 2014 Funom Theophilus Makama