Question

Ch. 42 Case study

A 10-month-old girl was admitted to the hospital for cardiac catheterization. Her history included cyanosis noted at about 6 weeks of age, increasing over the last 7 months and becoming more severe with crying or physical activity. A presumptive diagnosis of tetralogy of Fallot (TOF) was made on admission.

1. What is tetralogy of Fallot (TOF)?

2. What other conditions may produce cyanosis in the first year of life?

3. What are hypercyanotic spells? How are they treated?

4. What techniques or surgical procedures are available for treating this patient?

Answer 1

1.TETRALOGY OF FALLOT

TOF is the most common cyanotic congenital heart disease.This condition is characterized by the cambination of four defects:pulmonary stenosis,ventricular septal defect,overriding or dextroposition of the aorta and right ventricular hypertrophy.

2.OTHER CONDITIONS CAUSES CYANOSIS IN THE FIRST YEAR OF LIFE.

Transportation of great ateries-It occurs when the pulmonary arteries orginated from the left ventricle and aorta orginates from the right ventricle.It is an embryological defect and it is the most important cause of cyanosis at birth and responsible for most of the mortality from cyanotic congenital heart disease.

Tricuspid Atresia-It is the congenital absence of tricuspid valve resulting in no communication between right atrium and right ventricle.So the total systemic venous return enters the left heart menns of foramen ovale or an ASD,resulting in cyanosis.

Truncus arteriosus,Hypoplastic left heart syndrome,Total anomalous pulmonary venous return,Eisenmenger syndrome or complex are also causing cyanosis

3.HYPERCYANOTIC SPELL

It is a major clinical manifestation of tetralogy of fallot.It is also called blue spell.It occur due to cerebral anoxia.The spell consist of irritability,dyspnea,cyanosis,flacidity with or without unconciousness.It is commonly found in the morning soon after awkening,during or after feeding and painful procedures.

Management of hypercyanotic spell

*Place baby in knee-chest position.

*Sedatives

*Oral propranolol therapy

*Administration of IV vasopressors

*Oxygen therapy

SURGICAL PROCEDURES FOR PATIENT WITH TETRALOGY OF FALLOT

Surgical procedure can be performed as palliative surgery or definitive correction

Techniques of palliative surgery

*Modified Blalock-Taussing(BT) shunt

*Potts operation or Waterson's operation.

Definitive surgery is performed by direct vision open heart surgery for patch closure of VSD and relief of right ventricular obstruction.