overviewofcongenitalhertdisease-141101114226-conversion-gate02.pptx

overviewofcongenitalhertdisease

1. Overview Of Congenital Heart Disease Dr. SEHAR ZAHID MBBS, FCPS, PGPN, CHPE

2. Fetal Cardio-vascular circulation

3. Blood from Placenta Inferior Vena Cava Right atrium Right ventricle Pulmonary artery Aorta via ductus arteriosus Lower extremities Blood to placenta Left atrium via foramen ovale Left ventricle aorta Head and neck Returns via superior vana cava Right atrium

4. Congenital heart disease (CHD) is the most common congenital disorder in newborns. Leading causes of infant mortality. Urgent consultation/referral to a pediatric cardiologist

5. Duct – dependent lesions Coarctation of the aorta (COA) Interrupted aortic arch Aortic stenosis Hypoplastic left heart syndrome (HLHS) Transposition of the great arteries

6. Duct Dependent Circulation To Supply Systemic Circulation : Interrupted Aortic Arch CoA HLHS To Supply Pulmonic Circulation : Critical PS Pulmonary Atresia Severe Ebstein Anomaly For Mixing of Circulation : TGA with Intact IVS

7. Shock In Left heart obstructive lesions (e.g., HLHS, Critical AS, CoA , and Interrupted AA), systemic perfusion is lost. In Right-sided obstructive lesions (e.g., TAPVC, TA, and Mitral Atresia), restricted pulmonary blood flow results in reduced systemic blood flow, which may result in shock. In lesions with parallel pulmonary and systemic circulations (e.g., TGA with intact ventricular septum), mixing between the two circulations is decreased, leading to hypoxia and metabolic acidosis, which results in failure and shock.

8. Cyanosis Cyanosis is the bluish discoloration of the skin that occurs from the presence of deoxygenated hemoglobin (which is blue) in capillary beds. For cyanosis to be clinically apparent, 3 to 5 milligrams/ dL of Deoxyhemoglobin must be present, corresponding to an oxygen saturation of 70% to 80% on room air. Congenital heart defects that present with cyanosis include TGA, TOF, TA, Truncus arteriosus, and TAPVR. These lesions have in common the mixing of Oxygenated and Deoxygenated blood, circulation of Desaturated hemoglobin, and a cardinal manifestation as Cyanotic heart disease. Another condition resulting in cyanosis is persistent fetal circulation, which can be caused by structural heart disease or Noncardiac disease, including Meconium aspiration, pneumonia, sepsis, and pulmonary hypertension.

9. Cyanosis in Nonductal-dependent congenital heart defects : Total anomalous pulmonary venous connection (TAPVC). Truncus arteriosus. Lesions may or may not be ductal dependent depending upon the degree of outflow tract obstruction including tetralogy of Fallot and tricuspid atresia. Other lesions may exhibit differential cyanosis, such as critical Coarctation of the aorta or interrupted arch, where the deoxygenated flow through the ductus supplies the lower half of the body's circulation, but oxygenated blood flow from the left heart supplies the upper body via the vessels proximal to the site of arch obstruction.

10. Cyanosis

11. Severe Pulmonary Edema Pulmonary edema, resulting in tachypnea and increased work of breathing, can occur when there is a massive, rapid increase in pulmonary blood flow associated with a fall in pulmonary vascular resistance at delivery. In conditions such as Truncus arteriosus or PDA in premature infants, or pulmonary venous circulation obstruction in total anomalous pulmonary venous connection with obstruction.

12. Vitals Pulse : Abnormal heart rate — In infants with heart rates that are higher or lower than the normal range of 90 to 160 beats per minute for neonates up to six days of age, electrocardiography is initially performed to determine whether there is an arrhythmia. Respiratory rate : Tachypnea (>40/min) Screening according to AAP,AHA,ACCF (1 out of 3) : SpO 2  measurement <90 percent SpO 2  measurement <95 percent in both upper and lower extremities on three measurements, each separated by one hour SpO 2  difference >3 percent between the upper and lower extremities

13. On Examination CVS Auscultation Split S2 Early systolic clicks Mid-systolic clicks S3 gallop Pericardial friction rubs Murmurs  Innocent / Pathologic/ Absent Peripheral Pulses Examination & BP diff > 10 mm of Hg Coughing & Wheezing : Pulm . Vs. Cardiac involvement

14. History Maternal and prenatal history Preterm infants (gestational age <37 weeks): CHD is two to three times that found in term infants Maternal conditions that increase the risk of neonatal CHD include the following: Diabetes, obesity, hypertension, CHD – refer to family history, Thyroid conditions Epilepsy and mood disorders Maternal fever or influenza Smoking in the first trimester Congenital complete heart block in offspring of mothers with connective tissue disorders and anti-Ro/SSA and anti-La/SSB antibodies. Congenital infections such as cytomegalovirus, herpesvirus , rubella, or coxsackie virus.

15. Drugs taken in pregnancy such as Hydantoin  PS, AS Lithium  Ebstein's anomaly Alcohol  ASD, VSD Assisted reproductive technology (ART) increases the risk for congenital heart disease, particularly for malformations of the outflow tracts and ventriculo -arterial connections. It is unclear if this risk is related to the underlying etiology of infertility in the couple or the ART per se.  Family history  — There is an overall threefold increased risk for CHD when a first degree relative has CHD. The familial risk of specific malformations is even greater, suggesting a stronger genetic effect in these conditions

16. Reference: http://www.uptodate.com/contents/congenital-heart-disease-chd-in-the-newborn-presentation-and-screening-for-critical-chd#H3215187 Tintinalli’s Emergency Medicine A Comprehensive Study Guide, 7th Edition

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