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PJB

dr Amaliah Harumi Karim

syok .3 tanda penting yang perlu diperhatikan pada bayi PJB • Sianosis • Penurunan perfusi sistemik • Takipnea • BBL dengan PJB biasanya mempunyai 1 dari 4 gejala ini = murmur asimptomatik. sianosis. HF.

dan simtomatik . • Bising  bisa asimtom. Terdengar dalam jam2 pertama.Neonatus dengan murmur asimptom • Bising transient  akibat aliran darah pada duktus arteriosus yang sedang menutup.

Lesi stenotik katup pulmonal simtomatik Lesi stenotik katup aorta bising VSD asimptomatik AVSD .

although much of this output is ineffective mediated by an increase in sympathetic nervous oxygen consumption. however. total To maintain this high level of left with the left ventricular output is actually several times greater ventricular output. pulmonary symptoms of sweating and irritability and the vascular resistance imbalance between eventually begins to rise and. by several years of age. oxygen supply and demand lead to failure to thrive the shunt volume will decrease and eventually reverse to right to left . combined The term heart failure is a misnomer. often beyond the oxygen because it system activity transport ability returns directly to the lungs. Remodeling of the heart occurs. If left untreated. Lanjutan catatan The increase in circulating catecholamines. heart rate and stroke volume are increased work of breathing. with predominantly dilatation and a lesser Sympathetic activation leads to the additional degree of hypertrophy. total body normal. of the circulation. results in an elevation in than increased.

the combination of a left-to-right shunt with AV ( Chapter 433 ) valve regurgitation increases the volume load on the heart and often leads to more severe symptoms. endocardial cushion defects). . Regurgitation through the AV valves is most commonly encountered in patients with partial or complete AV Additional lesions that impose a volume load on the heart septal defects (AV canal. In include regurgitant lesions ( Chapter 422 ) and the these cardiomyopathies lesions.

occur at cardiac output will be maintained and the valve.Lesions Resulting in Increased Pressure Load The most frequent are obstructions to Less common are obstruction to The pathophysiologic common ventricular outfl ow: valvular pulmonic ventricular infl ow: tricuspid denominator of these lesions is stenosis. and coarctation obstruction of the of the aorta pulmonary veins Ventricular outfl ow obstruction can Unless the obstruction is severe. below the valve (double- the clinical symptoms of chambered right ventricle. . heart failure will be either subtle or subaortic membrane). or mitral stenosis. or above it absent (branch pulmonary stenosis or supravalvular aortic stenosis). cor triatriatum and an obstruction to normal blood fl ow valvular aortic stenosis.

.• This compensation • predominantly involves an increase in cardiac wall thickness • (hypertrophy). but in later stages it also involves dilatation and • can progress to ventricular dilation and failure.

peripheral edema). Severe pulmonic stenosis in the newborn period (critical • pulmonic stenosis) results in signs of right-sided heart failure • (hepatomegaly. as well as cyanosis from rightto- • left shunting across the foramen ovale . which is usually encountered in the immediate newborn • period. The infant may become critically ill within several hours • of birth.• The clinical picture is different when obstruction to outfl ow • is severe.

. peripheral edema). but not to cyanosis unless a pathway persists • for right-to-left shunting (e. In older • children. patency of the foramen ovale).• Severe aortic stenosis • in the newborn period (critical aortic stenosis) is characterized • by signs of left-sided heart failure (pulmonary edema. • and it may progress rapidly to total circulatory collapse. poor perfusion) • and right-sided failure (hepatomegaly..g. severe pulmonic stenosis leads to symptoms of rightsided • heart failure.

In these • patients. when the ductus • fi nally closes. • however. In the immediate newborn period. These infants • then become symptomatic. . the open aortic end of the ductus may serve as a conduit • for blood fl ow to partially bypass the obstruction. usually within the 1st 2 mo of life. often dramatically. the clinical presentation of coarctation may be delayed • because of the presence of a patent ductus arteriosus.• Coarctation of the aorta in older children and adolescents is • usually manifested as upper body hypertension and diminished • pulses in the lower extremities.

Atrial Septal Defect .

primum. or sinus venosus) .ASD • Atrial septal defects (ASDs) can occur in any portion of the atrial septum (secundum.