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A 30-year-old woman, who recently emigrated from Guatemala, presents to the ED with progressive dyspnea on exertion. There is a continuous machine like murmur that is heard in systole and diastole.
patent ductus arteriosis (PDA)
How do you diagnose PDA?
Definitive diagnosis of PDA should be based on visualization by imaging techniques and demonstrations of the shunting across the defect (with or without evidence of clinically significant LV volume overload).
What are ways to close PDA?
duct occluder (98% success rate)
coil closure
What are long term complications of PDA?
Eisenmenger syndrome
differential cyanosis
Heart Failure due to the left to right shunt.
Infective Endocarditis
Pulmonary hypertension
Aneurysmal dilation of PA, calcification of PA
Rupture of PA.
In adults born with a large left-to-right shunt through the ductus arteriosus, pulmonary vascular obstruction with pulmonary hypertension, right-to-left shunting, and cyanosis have usually developed
eisenmenger syndrome
Severe pulmonary vascular disease results in reversal of flow through the ductus; unoxygenated blood from the PA is shunted to the descending aorta; hence the toes—but not the fingers—become cyanotic and clubbed
differential cyanosis
When is closure recommended for PDA?
LA and/or LV enlargement or if PAH is present, or in the presence of net left-to-right shunting.
Prior endarteritis.
Careful evaluation and consideration for calcified PDA
When is surgical closure recommended for PDA?
The PDA is too large for device closure.
Distorted ductal anatomy precludes device closure (eg, aneurysm or endarteritis)
If patient has small PDA without evidence of left-sided heart volume overload, what is the proper management?
follow up every 3-5 years
When is endocarditis prophylaxis contraindicated?
patients with a repaired PDA without residual shunt
A 32-year-old woman presents with her fifth bout of pneumonia over the past 5 years.
She never smoked and had a normal pregnancy when she was age 28.
She states she has had chronic cough and dyspnea for months and symptoms worsen with exertion.
On exam, her BP is 120/70. Heart exam reveals a widely split S2 that does not change with respiration;
there is 3/6 mid-systolic murmur at the pulmonic valve area.
atrial septal defect (ASD)
Explain the mechanism of the wide fixed split of S2 in ASD
Due to the left to right shunt the RV-PA flow increases. This delays P2. (Wide split)
When the patient takes a deep breath, venous return to RA-RV increases, but the LA to RA shunt decreases also, making the volume going to PA the same. Hence split remains same (fixed)
What is the treatment for ASD?
percutaneous or surgical closure
when is ASD closure contraindicated?
severe irreversible PAH
no evidence of left to right shunt
When is surgical closure, but not percutaneous closure indicated for ASD?
sinus venosus ASD
coronary sinus ASD
primum ASD
anatomy precludes use of percutaneous device
concomitant surgical repair/replacement of tricuspid valve
What is the management of patients with chronic atrial tachyarrhythmias with ASD?
concomitant maze procedure
An 18-year-old male athlete presents for a sports physical examination to your office.
On cardiac auscultation you notice a harsh grade IV crescendo-decrescendo murmur at the left sternal border which increases on standing and Valsalva maneuver and decreases on sustained hand-grip.
hypertrophic cardiac myopathy
Heterogenous Disease with Autosomal Dominant Inheritance
Overall 2/3 of pts have a familial Hx , presents in early adulthood
HCM
A 30-year-old Hispanic man with a history of congenital heart problems was admitted for symptoms of heart failure.
He is not currently being followed by a cardiologist and he is unsure of the details of his heart condition. He reports that he has episodes where he feels very short of breath, his lips turn blue and squatting helps his symptoms.
Physical exam is significant for clubbing of the digits and a pulmonary systolic murmur. A chest x-ray, ECG and a transthoracic echocardiogram were performed.
tetralogy of fallot
Explain the boot shaped heart seen on chest x-ray in tetralogy of fallot
RVH --> uplifting of cardiac apex and
small pulmonary arteries --> concave main pulmonary artery segment)
describe heart abnormalities in tetralogy of fallot
Infundibular stenosis.
Over riding Aorta
Large VSD
RVH
What is a characteristic of the extremities in tetralogy of fallot?
cyanotic clubbing of hands
What is the management of tetralogy of fallot?
widening of pulmonary artery above (with patch) and below the valve
VSD repair (with patch)
What are the long term complication of tetralogy of fallot?
Severe pulmonic Regurgitation
RV failure
Arrhythmias – Atrial & Ventricular.
Endocarditis
Aortic root problems.