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Congenital Acyanotic Heart Disease

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General Principles

General: Systemic flow is well oxygenated via the pulmonary circulation. With time pulmonary hypertension can alter flow and shunts and result in reduced pulmonary flow and cyanosis. This is called Eisenmenger's syndrome
Causes: The causes are a mixture of causes with Shunts: VSD, ASD, PDA, Partial Anomalous venous drainage and without Shunts: Aortic coarctation, Hypoplastic left heart, Congenital Aortic stenosis
Pulmonary Flow is increased with VSD, ASD, PDA, aortopulmonary window, ruptured aneurysm of Valsalva, coronary artery fistula, partial anomalous pulmonary venous return (PAPVR) and normal with small shunts (see above), aortic valve stenosis, aortic coarctation, pulmonary stenosis
Clinical: reduced cardiac output with heart failure, breathlessness, tachycardia, Failure to thrive, murmurs, arrhythmias, cardioembolism
Investigations: FBC, U&E, ESR, ECG/EKG: RBBB, LBBB and axis deviation as well as tachycardia and arrhythmias. CXR: look for pulmonary plethora with the increased pulmonary flow. Look at the heart size and shape. Echocardiogram is vital and shows structural defect anatomy. may need Catheter studies
Management: see below

Condition	Clinical Findings	Investigations	Management
Atrial Septal Defect (ASD)	Often asymptomatic in childhood Fatigue, exertional dyspnoea in older children Fixed splitting of the second heart sound (S2) Systolic ejection murmur over the pulmonic area	Echocardiography (definitive diagnosis) Chest X-ray (cardiomegaly, increased pulmonary vascular markings) ECG (right axis deviation, right bundle branch block)	Small ASDs may close spontaneously Transcatheter device closure for moderate to large ASDs Surgical repair if transcatheter closure is not feasible
Ventricular Septal Defect (VSD)	Heart murmur (holosystolic) heard best at the left lower sternal border Symptoms depend on the size of the defect Small VSD: often asymptomatic Large VSD: Heart failure symptoms, failure to thrive, frequent respiratory infections	Echocardiography (to assess the size and location of the defect) Chest X-ray (cardiomegaly, increased pulmonary vascularity in large VSDs) ECG (left atrial enlargement, left ventricular hypertrophy)	Small VSDs may close spontaneously Diuretics, ACE inhibitors, and digoxin for heart failure symptoms Surgical repair for large VSDs or if symptomatic despite medical therapy
Patent Ductus Arteriosus (PDA)	Continuous "machine-like" murmur heard best at the left infraclavicular area Wide pulse pressure Bounding pulses In large PDAs: heart failure symptoms, respiratory distress	Echocardiography (visualization of PDA and its haemodynamic impact) Chest X-ray (cardiomegaly, increased pulmonary vascularity) ECG (usually normal, left atrial enlargement in large PDAs)	Indomethacin or ibuprofen to close the PDA in preterm infants Transcatheter device closure or surgical ligation for persistent PDA Monitoring for complications such as endocarditis
Coarctation of the Aorta	Hypertension in the upper extremities Weak or delayed femoral pulses Blood pressure discrepancy between arms and legs Heart murmur (systolic) heard over the back or left axilla	Echocardiography (definitive diagnosis) Chest X-ray (rib notching, "3 sign" indicating indentation of the aorta) CT or MRI angiography (to assess the severity and extent of the coarctation)	Balloon angioplasty with or without stenting Surgical repair for severe cases or if angioplasty is not successful Long-term follow-up for monitoring hypertension and restenosis
Aortic Stenosis	Systolic ejection murmur heard best at the right upper sternal border Syncope, angina, and dyspnoea on exertion in severe cases Narrow pulse pressure Left ventricular hypertrophy signs	Echocardiography (to assess the severity of stenosis and left ventricular function) ECG (left ventricular hypertrophy, strain patterns) Chest X-ray (may show post-stenotic dilation of the aorta)	Balloon valvuloplasty for symptomatic or severe stenosis Surgical aortic valve replacement for critical cases Long-term monitoring and management of complications such as endocarditis
Pulmonary Stenosis	Systolic ejection murmur heard best at the left upper sternal border Right ventricular hypertrophy signs Exertional dyspnoea Cyanosis in severe cases	Echocardiography (to assess the severity of stenosis and right ventricular function) ECG (right ventricular hypertrophy) Chest X-ray (may show post-stenotic dilation of the pulmonary artery)	Balloon valvuloplasty for symptomatic or severe stenosis Surgical repair if balloon valvuloplasty is unsuccessful Long-term follow-up for monitoring potential complications

PLEASE NOTE LEGAL ADVICE: The contents are under continuing development and improvements and may contain errors of omission or fact. Feedback vital and always welcome at drokane at gmail.com. This is not to be used for the assessment, diagnosis or management of patients. It should not be regarded as medical advice. It is only for educational purposes. Please adhere to your local protocols. If you are unwell please seek healthcare advice from your doctor. This does not replace senior or specialist advice. If you do not accept this then please do not use the website. If you need medical advice, please consult a doctor or other appropriate medical professional. If you are a medical professional and you need advice then speak to your senior or colleagues.

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