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Congenital Heart Dis

Congenital Heart Disorders

QuestionAnswer
At what time do you expect to see heart tube morphogenesis abnormalities? 4-7 weeks
Down Syndrome: genetic cause; symptoms; incidence; most common defects trisomy 21; hypotonia and developmental delays; 40-50% incidence (most common); AV septal or ventricular septal defects
Turner's syndrome: genetic cause; commonly seen defect Female XO karyotype; highly associated with coarctation of the aorta of the aorta bicuspid aortic valve
William's syndrome: genetic cause; commonly seen signs, symptoms, and defects Elestan gene (7q11); developmental delya; loquacious personality and facial dysmorphology; supravalvular aortic stenosis, pulmonary artery stenosis, and renal artery stenosis
Noonan's syndrome: genetic cause; signs; cardiac abnormalities autosomal dominant genetic abnormality (12q); similar to Turner's phenotype: short stature, web neck, hypertelorism, and developmental delay; cardiac abnormalities: pulmonary valve stenosis and hypertrophic cardiomyopathy
What is a secundum defect? Most common of atrial septal defect, where there is a shunt from the left to right atrium near the region of the fossa ovalis
What is a sinus venosus type defect? Atrial septal defect adjacent to the superior vena cava or to the inferior vena cava called?
What clinical findings do you see in patients with atrial septal defects? prominent parasternal impulse, fixed splitting of the 2nd heart sound, an ejection murmur a the mid to upper left sternal border and usually a mid-diastolic murmur at the low left sternal border
What is the physiological cause for the murmur heard during diastole in a patient with an atrial septal defect? Increased flow across tricuspid and pulmonic valves resulting in (relative) tricuspid and pulmonic stenosis-->high velocity flow
What x-ray findings do you see in patitents with atrial septal defects? Cardiomegaly with increased pulmonary vascular markings
What ECG findings do you see with an atrial septal defect? Right axis deviation and right ventricular hypertrophy
What is the diagnostic imaging modality of choice for a person with an atrial septal defect? Echocardiography; shows the size and type of defect as well as the nature of the shunting
What are some possible complications due to an atrial septal defect? Children rarely symptomatic; symptoms as adults: right HF (due to volume overload), atrial arrhythmias (from atrial stretching), pulmonary hypertension, paradoxical emboli from right to left atrial flow (rare)
What is the treatment for atrial defects? Elective clousure by age of 3-4 years; most secundum effects can be treated by a transcatheter approach avodiing the need for open heart surgery
what is a doubly committed/supracristal defect? An AV septal defect that is adjacent to the aortic and pulmonary valves
What dtermines the severity of an AV septal defect size of the holw and the down stream resistance to flow
What clinical findings do you expect with a small VSD? Normal intracdic pressures: No symptoms; high pitched, pansystolic murmur low left sternal border, normal S2
What clinical findings do you expect with a large VSD? Pulmonary hypertension: congestive heart failure; failure to thrive in infancy; ejection murmur, loud single S2
What XR results do you see in patients with VSD? ECG? What is diagnostic? XR: large heart, inc. vascularity; ECG: LVH +/- RVH; echocardiogram--diagnostic; catherization infrequently necessary)
What is the prognosis for patients with VSDs? What are possible complications? How should they be treated/managed? Spontaneous closure or decreased in size common; congestive heart failure, failure to thrive, pulmonary hypertension, vascular diseases, bacterial endorcarditis; control of HF and preventrion of pulmonary vascular diseases
What is the difference between a complete and a partial AV septal defect? Complete: contiguous atrial and ventricular communications with a large left-to-rigth shunt + volume overload of the RA, RV, and LV; partial: no intraventricular communication, low atrial defect along with abnormal AV valves
What genetic disorder are AV septal defects common with? Down syndrome
What signs are seen in patients with complete AV septal defects? Findings of large ventricular septal defect including pulmonary hypertension and congestive heart failure
What signs are seen in patients with partial AV septal defects? Partial defects will present similar to an atrial septal defect associated with mitral regurgitation
What are the long term complications associated with an AV septal defect? AV valve regurgitation, congestive heart failure, pulmonary hypertension and eventually pulmonary vascular disease and endocarditis
What is the treatment for patients with AV septal defects? surgical closure of the defect(s)
What hemodynamic abnormalities are associated with a patent ductus arteriosus? left to right shunting leads to volume overload of the left atrium and left ventricle
What are the clinical findings seen in a patent ductus arteriosus? Continous murmur at the upper left sternal border and bounding pulsus (result of diastolic run off from the aorta to the pulmonary artery)
What complications are seen in patients with patent ductus arteriosus? Congestive heart failure, pulmonary hypertension, and endocarditis
What's the prognosis for patients with PDAs? Most can be closed off in the catheterization laboratory with a transcatheter coil or device placement
What is Eisenmenger's syndrome? Longstanding pulmonary hypertension-->damage to pulmonary vasculature at the arteriolar level-->progressive increase in pulmonary vascular resistance over time-->PA pressure > systemic pressure --> shunt shifts to right-to-left-->CYANOSIS
What physiological changes are seen in patients with pulmonic stenosis? Elevated pressure in right ventricule-->right ventricular hypertrophy
What clinical findings do you see in patients with pulmonic stenosis? Ejection click, harsh sytolic ejection murmur at the upper left sternal border; ECG will show right ventricular hypertrophy and the chest XR shows dilated main pulmonary artery (due to post-stenotic dilation)
What are the main complications due to pulmonic stenosis? Right heart failure, cyanosis (if there is a right to left atrial communication shunt)
How is pulmonic stenosis typically treated? With a transcatheter ballon (dilation)
What physiological changes do you see in patients with aortic stenosis? Obstructed outflow-->increased pressure (afterload) in LV-->hypertrophy
What clinical findings do you see in patients with aortic stenosis? Systolic ejection murmur at the right upper sternal border; apical ejection sound; decreased upstroke of the carotid and peripheral pulses (if lesion is severe); ECG: left ventricular hypertrophy; echo is diagnostic
What is the treatment for patients with aortic stenosis? Transcatheter balloon dilation is usually effective only in a palliative procedure; most patients eventually required aortic valve later in childhood or adulthood
What are complications seen in patients with aortic stenosis? congestive heart failure; sudden death; exertional syncope; endocarditis
What are the complications associated with coarctation of the aorta? decreased blood pressure --> cardiogenic shock; severe congestive heart failure
Created by: karkis77 on 2011-09-22



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