Learning Objectives:

Identify and describe specific acyanotic cardiac anomalies. Explain the pathophysiologic effect for each lesion. Identify and explain circumstances under which acyanotic lesions become cyanotic. List the clinical signs for acyanotic lesions, and explain how diagnosis is made. Describe the treatments for each condition, including surgical repair.

Acyanotic Cardiac Anomalies Respiratory Associates of Texas

Acyanotic anomalies- types

Non-obstructive Septal defects Conduction defects Obstructive Stenosis Coarctation

Non-obstructive Acyanotic Anomalies

Consequences of anomalies
Obstruction ==> Reduced flow Increased ventricular afterload

Consequences
Septal defects L R shunt (normal PVR
Increased LV work ==> CHF Chronic pulmonary infection Pulmonary vascular dx ==> R L shunt ==> hypoxemia

Conduction defects ==> arrhythmias

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Ventricular Septal Defects

Small VSD (less than diameter of aorta) L R shunt if VSD < 50% aortic diameter RV & LV pressures normal May close spontaneously

Small VSD
Manifestations may be asymptomatic only clinical sign may be murmur other data normal

Small VSD with L-R shunt

Large VSD = VSD diameter > aorta

Hemodynamics L R shunt > 50% ==>
LV work ==> CHF PA flow ==> Eisenmenger's complex PAP ==> R L shunt ==> hypoxemia

Large VSD with R-L shunt

Large VSD
Manifestations Murmur CHF Cyanosis when PAP is increased LV hypertrophy

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Large VSD
Diagnosis Heart catheter- high RV SaO2 Angiography- mixing Management Palliation- PA band Correction- patch, closure

PA banding- reduces pulmonary blood flow

PA bands

Atrial Septal Defects

Hemodynamics LAP > RAP ==> L R shunt ==>

ASD with L-R shunt

RA & RV SaO2 PA blood flow ==> Eisenmenger's complex RV failure (cor pulmonale)

Atrial Septal Defect

Manifestations May be asymptomatic for 20-30 Y First sign may be cor pulmonale CHF Murmur

Atrial Septal Defect

Diagnosis ECG - Right axis deviation Echo- enlarged Rt heart Heart catheter- high RA SaO2 Treatment- surgical closure

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Endocardial Cushion Defect

Pathogenesis- incomplete development of ECD Defects- permutations of: ASD VSD Cleft mitral, tricuspid valve leaflets

Endocardial Cushion Defect

Hemodynamics-- depend on specific defects Atrial L - R shunt Ventricular L -R shunt ==> LVH Mitral regurgitation ==> LAH SaO2 in RA & RV increased Increased PA flow ==> Eisenmenger's complex

Endocardial cushion defect

(Complete A-V canal) cleft AV valve leaflets

Endocardial Cushion Defect

Manifestations Asymptomatic CHF-- pulmonary edema Diagnosis ECG-- LAD Heart catheter- increased SaO2 in RA & RV

ASD VSD

Endocardial Cushion Defect

Treatment Palliative-- PA band Complete correction

Patent Ductus Arteriosus

Second most common anomaly in term infants Etiologic factors neonatal asphyxia, hypoxemia maternal viral infections, e.g., rubella low socioeconomic status- nutrition Note- patent ductus is necessary for survival in patients with ductaldependent anomalies

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Patent Ductus Arteriosus

Complications excessive workload on left ventricle pulmonary artery disease chronic pulmonary infections Manifestations persistent murmur decreased lung compliance==> increased work of breathing cardiomegaly diagnosed by echocardiogram

Patent Ductus Arteriosus

Management Medical
Indomethacin to close ductus Intubate and ventilate with PEEP

Surgical
ligation (sometimes done in NICU) division- requires thoracotomy

Aortic stenosis
Narrowed aorta or aortic valve Hemodynamics- increased resistance to LV outflow ==> increased LV work ==> hypertrophy ==> LV failure Manifestations Ejection systolic murmur LVH, LAD CHF, sudden death

Obstructive Anomalies

Management Avoid exercise

Valvotomy Valve replacement

Coarctation- narrowing of portion of aorta

Hemodynamics- obstruction Associated with Turner's syndrome Manifestations- depends on location Reduced pulses in lower extremities Headaches, leg cramps, epistaxis LVH, RVH Lower body cyanosis (with PDA) Management avoid heavy exercise resection- may require graft

Coarctation of aorta (pre-ductal)

Coarctation

venous admixture PDA

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Coarctation of aorta (post-ductal)

Coarctation

Conduction defect-- Wolfe-ParkinsonWhite syndrome

Impulse aberrantly conducted through bundle of Kent Manifestation-- paroxysmal atrial tachycardia (PAT) Treatment Medical- antiarrhythmics Surgical-- ablation of Bundle

PDA

References
Barnhart SL, Cervinske, MP. Perinatal and Pediatric Respiratory Care 1995. WB Saunders Company, Phila. Levin DL, Morriss FC. Essentials of Pediatric Intensive Care (volume one) 1997. Churchill-Livingston, NY. Johnson KB, Oski FA. Oski's Essential Pediatrics 1997. Lippincott-Raven, Phila.

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