Cyanotic Heart Defects: Cyanotic and Acyanotic

Source:  Cyanotic Heart Defects: Cyanotic and Acyanotic    Tag:  acyanotic congenital heart disease
Resident: Derek Nobrega
Title: Cyanotic Heart Defects: Cyanotic and Acyanotic
Authors: Soraya Nouri, MBBS, FACC
Journal: Pediatric Annals

Main Purpose:
To discuss the clinical manifestations of congenital heart disease.

Acyanotic Congenital Heart Disease
Left to Right Shunt Lesions
- Volume overload distal to the shunt and secondary congestive cardiac failure, manifesting as porr feeding, dyspnea, pulmonary congestion, and recurrent pneumonia.
Obstructive Lesions
- Examples = pulmonary stenosis, aortic stenosis, peripheral pulmonary stenosis, and coarctation of the aorta.
- Causes pressure overload and hypertrophy of the proximal chamber.
Primary Pump Failure
- occurs in dilated cardiomyopathy, anomalous origin of a coronary artery from the pulmonary artery, myocarditis, and severe congestive cardiac failure due to other myocardial diseases.
- Infants present with tachypnea, tachycardia, cardiac enlargement, respiratory distress, and acidosis.
 Left to Right Shunt with an Obstruction
- Right-Sided Obstruction = right sided venous congestion and right heart failure
- Distal Left-Sided Obstruction = VSD and coarctation of the aorta
- Proximal Left-Sided Obstruction

Cyanotic Congenital Heart Disease
Abnormal Communication Proximal to Right-Sided Obstruction
- Tetralogy of Fallot and Tricuspid Atresia with ASD
Abnormalities of Alignment Between Various Segments
- D-transposition of the great arteries, truncus arteriosus, and anomalous pulmonary venous return.
- Result in cyanosis with or without increased or decreased pulmonary blood flow.

Neonates and Infants
- The majority of patients who have severe CHD present during the first year of life, especially during the neonatal period.
- Cyanosis caused by deoxygenated blood in the systemic capillaries with at least 5g Hg in the blood
- Clubbing – significant and prolonged cyanosis causes clubbing of the nails
- Tachypnea and Dyspnea = tachynpnea is repiratory rate > 60 breaths/minute. Severe dyspnea in infant presents with grunting and subcostal retractions (respiratory distress).

Older Children
- Exercise intolerance
- Chest Pain
- Dysrhythmias
- Syncope and Sudden Death

Primary Tests for Diagnosis
- Electrocardiogram = diagnosis of dysrhythmias, assessment of chamber enlargement, hypertrophy, and myocardial ischemia
- Chest Radiograph = demonstrates pulmonary vascular pattern, cardiac size and contour, and associated lung or musculoskeletal abnormalities.
- Echocardiogram = detailed info about cardiac anatomy and hemodynamics
- Holter Monitor = determines whether dysrhythmia or ischemia is responsible for symptoms such as chest pain, dizziness, syncope, and dyspnea
- Exercise Testing = unravels latent dysrhythmias, ischemia, significant cardiac decompensation, and inadequate cardiac output or reserve.