Overview Congenital heart disease (CHD) is the most common congenital disorder in newborns.
Classification
| Cyanotic (T's) | Acynotic (D's) |
| Tetralogy of Fallot | ASD |
| Transposition of the great vessles | VSD |
| Tricuspid atresia | AVSD |
| Truncus arteriosus | Patent Ductus Arteriosus |
| Other: Pulmonary stenosis, Aortic stenosis, Coarctation of the aorta |
Epidemiology
Clinical Manifestation In infants, feeding difficulties may be the first sign of congestive heart failure
Cardiovascular
Constitutional
Respiratory
Differential Diagnosis - innocent murmur's
| Remember The most common innocent murmur is a Still murmur |
Investigations
Embryology
Foetal Circulation
Overview
Clinical Presentation
Investigations
Pathophysiology
Eisenmenger Syndrome (Cyanosis)
| Pulmonary hypertension is present when mean pulmonary artery pressure exceeds 25mm Hg at rest or 30 mm Hg with exercise. ECG findings include: RVH or strain, RAD, p-pulmonale, tall R waves in V1. |
Overview Most common congenital heart defect. It occurs in almost 50 percent of all patients with congenital heart disease.
Risk factors
Important features of VSD
| Remember 75% of small defects undergo spontaneous closure within the first two years of life. Moderate VSD may close overtime. Large rarely close and require surgery |
Clinical Presentation
| Causes of Pansystolic mumurs |
| Mitral regurgitation |
| Ventricular septal defect |
| Tricuspid regurgitation |
Pathophysiology of VSD
Eisenmenger Syndrome (Cyanosis)
Investigations and diagnosis
Management
Complications
Overview Normally ductus arteriosus closes and become ligamentum arteriosus. Patent ductus arteriosss is where the ductus arteriosus persists post partum for more than 3months. Patent ductus arteriosus creates a Left to right shunt. This occurs in 1 in 2000 live births.
Anatomy
The ductus arteriosus closes in approximately 90 percent of full-term neonates by 48 hours of age. Prolonged patency of the ductus arteriosus occurs in premature infants and those born at altitude
Risk Factors
Clinical presentation
Management
Overview A stricture is present in the aorta which leads to restricted blood flow to the lower part of circulation. BP in head and UL’s is high, whilst that in the LL’s is low. Heart failure may develop Surgical repair is needed in most cases.
Clinical presentation
| Remember Coarctation should be suspected in an asymptomatic child with hypertension |
Investigations
Management
Complications
Overview Aortic valve is thickened and narrowed leading to
development of abnormally high pressure in LV. LV becomes
hypertrophied. Correction with baloon valvuloplasty or surgery.
Pulmonary stenosis Pulmonary valve is thickened and narrowed leading to development of abnormally high pressure in RV. RV becomes hypertrophied If severe, treated with balloon catheter to dilate narrowing.
Overview The most common cyanotic congenital cardiac defect. In 1888, Etienne-Louis Arthur Fallot described four cyanotic patients with four similar anatomic features
4 anatomical features
| Side Note there are a number of associated anatomical features associated with TOF including right aortic arch, left anterior descending from the right coronary artery, patent ductus arteriosus. |
Pathophysiology
| Side note Children with cyanotic congential heart disease often squat. This kinks the femoral artery increasing peripheral resistance. This in turn increases left-sided ventricular resistance which lessens the burden of right-eft shunt. |
Clinical presentation - depends on degree of right ventricular outflow obstruction
Clinical Examination
Diagnosis
| Remember ECG findings of right ventricular hypertrophy include: p-pulmonale (Right atrium), tall R waves in V1, upright T wave in V1 and marked right axis deviation |
Management - surgical
Overview Eisenmenger syndrome is the triad of congenital systemic-to-pulmonary cardiovascular communication, pulmonary arterial disease causing severe pulmonary hypertension, and cyanosis
Diagnosis
Clinical findings
