DESSERT SESSION CYANOTIC CONGENITAL HEART DISEASE 1 2

DESSERT SESSION

CYANOTIC CONGENITAL HEART DISEASE 1. 2. 3. 4. CCHD with low PBF and no PAH CCHD with low PBF and PAH CCHD with high PBF CCHD with near normal PBF

CYANOTIC CONGENITAL HEART DISEASE CCHD with low PBF and no PAH 1. TOF 2. TOF equivalents (PS with VSD like pathology) 3. Pulmonary atresia with IVS 4. PS with ASD 5. Ebstein anomaly of TV

CYANOTIC CONGENITAL HEART DISEASE TOF equivalents A. DORV+ VSD+ PS B. D-TGA + VSD+ PS C. L-TGA + VSD+ PS D. Tricuspid Atresia + VSD + PS E. Single Ventricle + PS F. Truncus Arteriosus with small pulmonary arteries

CCHD with Low PBF & PAH Eisenmenger syndrome

CCHD WITH HIGHPBF Inter circulatory mixing (admixture physiology) Venous level: TAPVC Atrial level HLHS : Single Atrium, Tricuspid Atresia, Ventricular level : Single ventricle Arterial level: Truncus Arteriosus Transposition Physiology D TGA Taussing bing anomaly

CCHD WITH NEAR NORMAL PBF Ø Ø Ø Pulmonary Arterio Venous Fistula Anomalous drainage of vena cava to Left Atrium Un roofing of coronary sinus in to Left Atrium

CCHD CLASSIFICATION TOF physiology Transposition physiology Admixture physiology Pretricuspid- TAPVC, HLHS, TA, single Atrium Post-tricuspid- single Ventricle, TA Eisenmenger physiology Ductus dependent pulmonary circulation- PA Ductus dependent systemic circulation- HLHS Near normal physiology- Pulmonary AV fistula Miscelloneous- Ebstein anomaly, PS + ASD

CAUSES OF CYANOSIS AGE CAUSE OF CYANOSIS BIRTH 1. 2. 3. 4. 5. 1 st week 1. 2. 3. 4. 5. D TGA Pulmonary Atresia Tricuspid atresia Ebstein Critical PS > 1 week 1. 2. 3. 4. 5. 6. 7. TOF TGA Admixture lesions TAPVC SV DORV Truncus arteriosus D TGA Obstructive TAPVC Tricuspid atresia Pulmonary atresia with hypoplastic RV TOF (severe PS)

AGE FAILURECauses of Heart Failure HEART 1 ST day of life 1. 2. 3. 4. 5. 6. Large AV fistula Congenital severe PR/ severe TR Premature infant with Large PDA Critical AS Tachyarrthythmia/ bradyarrhythmia HLHS 1 ST week of life 1. 2. 3. 4. 5. Coarctation of Aorta Critical AS Critical PS Obstructed TAPVC HLHS 1 ST month of life 1. 2. 3. 4. 5. 6. Coarctation of Aorta with large PDA Large VSD Large PDA AV septal defect TGA with nonrestrictive VSD Truncus Arteriosus 6 month of life 1. 2. 3. 4. VSD with PDA ALCAPA Aortoventricular tunnels Any of the above conditions

Decreased PBF Presentation at Increased PBF Any age Neonate / Infant Appearance Comfortable Sick, Lethargic, Irritable Cyanosis Mild - Severe Mild (except TGA with intact IVS) Common Uncommon Feeding difficulty / � Sweating Absent Present Failure to thrive Absent Present Weight Gain Normal Suboptimal Recurrent LRI No Yes Tachypnea Absent Present Heart size Normal Cardiomegaly CHF, Tachycardia, S 3, S 4 Absent Present Olegemia, No Cardiomegaly Plethoric Lungs, Cardiomegaly Squatting /Cyanotic spells CXR

RECURRENT RESPIRATORY TRACT INFECTION 2 or more admissions in six months or three admissions for Pneumonia in any time frame ≥ 3 annual episodes of documented bronchitis, bronchiolitis, or pneumonia

LRTI IN CHD ↑ PBF Engorgement of pulmonary arteries Compress the adjacent bronchi and bronchioles Microatel ectasis Stasis of secretion s

LRTI IN CHD Goblet cell hyperplasia Increased mucus secretion

Structural defects of cilia or secondary to various infections Abnormalities of the respiratory mucus or defects in the mucociliary function Reduced ciliary movement Defects in clearance of airway secretions

Decreased immune mechanism (syndrome) blood pooling in lungs- bacterial growth