CONGENITAL HEART DISEASES I By Prof. Khaled KARARA Professor of Cardiothoracic Surgery Alexandria Faculty of Medicine

Classification CHD is a defect in the structure of the heart and great vessels which is present at birth. I-Acyanotic: A- With Shunt: -Ventricular Septal Defect (VSD). -Atrial Septal Defect (ASD). -Patent Ductus Arteriosus (PDA). B- Without Shunt: -Coarctation of Aorta (CoA). -Pulmonary Stenosis (PS). -Aortic Stenosis (AS). -Vascular ring.

Classification (cont.) II- Cyanotic: Tetralogy of Fallot (F4). Transposition of great arteries (TGA). Tricuspid Atresia. Truncus Arteriosus. Total Anomalous Pulmonary Venous Drainage. Eisenmenger’s Syndrome. Other: Hypo plastic left or right heart, Pulmonary Atresia (PA), Double Outlet Right Ventricle (DORV).

Clinical Picture Symptoms: No symptoms (accidentally discovered murmur). Shortness of breath, cyanosis and cyanotic spells, squatting position, syncope, poor feeding and failure to thrive, or repeated respiratory infections. Symptoms of associated defects.

Clinical Picture Signs: Follow the classical rules of general examination and local inspection, palpation, percussion and auscultation. Certain lesions have characteristic auscultatory findings: VSD → Pansystolic murmur max. in 3rd & 4th left spaces parasternally. ASD → wide fixed splitting of 2nd sound and ejection systolic murmur in pulmonary area.

Clinical Picture (Cont.) PDA → continuous machinery murmur in left upper parasternal area and wide pulse pressure. CoA → systolic murmur precordial and interscapular. Hypertension in upper extremity and weak or absent pulses in lower extremity. PS → harsh ejection systolic murmur in pulmonary area. AS → harsh ejection systolic murmur in aortic area.

Investigations ECG: Echo Doppler: Used to detect: Right ventricular Hypertrophy → ASD, PS, F4. Left ventricular Hypertrophy → PDA, AS. Biventricular Hypertrophy → VSD. Echo Doppler: Used to detect: Type and size of the defect. Cardiac size and function. Pulmonary pressure. Pressure gradient across the defect. Shunt ratio. Direction of blood flow.

Echocardiography Image shows a mid-muscular ventricular septal defect. The trace in the lower left shows the cardiac cycle and the red mark the time in the cardiac cycle that the image was captured. Colors are used to represent the velocity and direction of blood flow.

3D/4D echocardiography

Investigations (cont.) Chest X-ray: Pulmonary plethora → shunt (ASD, VSD, PDA) Pulmonary oligaemia → PS,F4 F4 → boot-shaped heart (Coeur en Sabot) CoA → figure 3 sign, rib notching. TGA → egg-shaped heart. Supracardiac type of TAPVC → Figure-of-eight (8) or Snow-man appearance.

Investigations (cont.) Multislice CT angiography (CTA): for CoA, Vascular rings and some cases of PDA. Cardiac MR (CMR) and Magnetic Resonance angiograghy (MRA): Cardiac Catheterization: Angiography to study well the anatomy. Take pressures to measure gradients. Take blood samples to measure O2 saturations. Therapeutic procedure may be done e.g. Atrial septostomy in TGA, Balloon dilatation in PS, AS & COA, Device closure in PDA,VSD & ASD.

CTA- Vascular Ring

CTA – Coarctation of Aorta

Coarctation of the Aorta- MRA

Cardiac Catheterization Diagnostic procedure

Non Surgical Treatment Medical: In infants; indomethacin and ibuprofen may be effective in closure of PDA & Prostaglandin E may be given to keep PDA open in cyanotic HD to improve oxygenation. Therapeutic catherization:

Surgical Treatment I- Closed cardiac procedure is required for: Complete Repair: PDA, CoA, Vascular ring. Palliative Procedure: Shunts for F4, Pulmonary artery banding. II- Open cardiac procedure is required for: Any procedure requiring opening of cardiac chamber e.g. ASD, VSD, total correction of F4.

Aim of Surgery for Congenital Heart Diseases 2 main categories: Palliative Procedures: To provide symptomatic relief, Providing an improvement in the patient’s O2 saturation and Permitting growth until the child has complete correction. Complete (Total) repair: A-Repair of extra cardiac anomalies: usually no need for cardiopulmonary by pass The commonest examples are PDA, Coarctation of aorta and Vascular ring. B- Repair of intra cardiac anomalies: Usually carried out using cardiopulmonary bypass. The commonest anomalies are VSD, ASD, and Tetralogy of Fallot.

Palliative Procedures Classic palliative procedures: Aortopulmonary shunt: Aiming to increase pulmonary blood flow Pulmonary artery banding: Reduces pulmonary blood flow aiming to avoid progression to irreversible pulmonary vascular disease. Glenn Cavo-pulmonary shunt: Connects the SVC to right pulmonary artery.

Modified Blalock-Taussig shunt (MBT Shunt) It consists of Interposition of a poly tetra fluoro ethelene (PTFE, GORE-TEX®) tube graft between the subclavian or innominate artery and the right or left pulmonary artery . It is the most commonly performed shunt procedure.

Classic & Modified Blalock-Taussig shunts