Cardiology #1 (Cardiomyopathies & Congenital Heart Disease) Flashcards
Explain the pathophysiology of dilated cardiomyopathy.
What are some causes of this (think of the 6D’s).
Also explain some symptoms.
Systolic dysfunction leading to a weak dilated heart. The ventricles cannot contract well –> poor EF
-The heart dilates to compensate for the weak ventricles (contraction problem)
EtOH, postpartum state, cocaine use, Doxirubicin, Vitamin B1 (thiamine) deficiency, Idiopathic (MC), Viral (Coxsackievirus), Radiation
Systolic heart failure symptoms: weakness, fatigue, SOB, dyspnea, orthopnea, DOE, (LHF). Peripheral edema, JVD, hepatomegaly (RHF).
S3 gallop and crackles.
What diagnostics are done for dilated cardiomyopathy and what do they show?
CXR: cardiomegaly, pulmonary congestion (fluffy infiltrates)
Echo (DOC): LV dilation, decreased EF, thin ventricular walls
ECG: LVH, tachycardia, arrhythmias (convex or inverted ST segment)
What kind of cardiomyopathy does alcohol consumption cause?
Dilated Cardiomyopathy
What are some health maintenance measures you can educate the patient on to avoid dilated cardiomyopathy?
Limit alcohol consumption
Control BP, lipids, and DM
Increase Exercise
Treat underlying disorder
What is the treatment for dilated cardiomyopathy?
Standard HF Treatment
-BASH
–BB
–ACEi
–Spironolactone
–Hydralazine + Nitrates
-Diuretics, Digoxin for symptoms
-Sodium restriction
-AICD if EF < 30%
-Cardiac transplant may be needed
What studies should you get for postpartum cardiomyopathy?
Echo and BNP (This is pregnancy related dilated cardiomyopathy)
What is stress (Takotsubo) Cardiomyopathy?
What is the pathophysiology behind this condition?
Transient systolic dysfunction of the LV that may imitate an MI but is in the absence of CAD or evidence of plaque rupture.
Catecholamine surge during times of extreme emotional or physical stress
What are the main risks of stress cardiomyopathy
Postmenopausal state
Exposure to physical or emotional stress
Symptoms of stress cardiomyopathy
-Substernal CP
-Dyspnea
-Syncope
-Indistinguishable from MI
For stress cardiomyopathy, often the cardiac enzymes are elevated. However, what does a coronary angiography show?
No plaque rupture or coronary artery disease
Treatment for stress cardiomyopathy
-Much like ACS: Aspirin, BB, Nitro, Heparin, and coronary angiography to rule out MI
-Short Term: Conservative, supportive due to transient nature
BB or ACEI for 3-6 months
Explain what restrictive cardiomyopathy is and what the main causes are
Diastolic dysfunction in a non-dilated ventricle which impedes ventricular filling (decreased compliance due to stiffness)
Infiltrative Disease: Amyloidosis (MC), Sarcoidosis, Hematochromatosis, Scleroderma, Fibrosis = deposits in the heart
-Also other causes are radiation and chemotherapy
Symptoms of Restrictive cardiomyopathy
-Right sided HF > Left sided HF
–Peripheral edema, JVD, Ascites, GI, hepatomegaly
–Dyspnea
–Kussmaul’s Sign: increase in JVP with inspiration
An echo can be done for restrictive cardiomyopathy. What is seen on an echo and what is seen if the cause is Amyloidosis?
Usually, non-dilated ventricles with normal thickness, diastolic dysfunction, and marked dilation of both atria.
Amyloidosis: bright speckled myocardium with amyloidosis
What is the definitive diagnostic for restrictive cardiomyopathy? What is seen with amyloidosis?
Endomyocardial biopsy
Amyloidosis: apple green birefringence with Congo Red stain
Treatment for restrictive cardiomyopathy
-Treat underlying disease
-Diuretics for symptoms can be given
In hypertrophic cardiomyopathy, what is the reasoning behind it? Also, it is what type of inheritance pattern and what is the mutation in that causes this condition?
Ventricles are too thick, impedes filling –> diastolic dysfunction (mostly LV)
Autosomal dominant pattern
Mutation in the sarcomere genes
Symptoms of HCOM
-Dyspnea (MC), Angina, Arrhythmias, sudden cardiac death during exertion
-Exercise induced syncope due to V-Fib
What does the murmur with HCOM sound like and why? What maneuvers make the murmur increase? Decrease?
Harsh systolic murmur at LSB
-Increased murmur intensity with decreased venous return (standing, Valsalva) = heart is smaller. The Aorta contracts and leads to more turbulent flow
-Decreased murmur intensity with increased venous return (leg raise, supine, and squatting) = bigger heart.
-Loud S4, pulsus bisferens (double pulse)
What does an echo for HCOM show?
EKG?
CXR?
Asymmetrical ventricular wall thickness (septal hypertrophy) 15 mm or more
LVH
Cardiomegaly
Treatment for HCOM? Medical vs Surgical
BB are first line (slow rate of contraction and rate)
AICD for ventricular arrhythmias
Myomectomy if young; alcohol septal ablation is an alternative
What are some recommendations for those with HCOM (things they should avoid, things they should do, etc.)
Avoid dehydration, exercise, exertion
Caution with Digoxin, Nitrates, and Diuretics (positive inotropes) because they lower LV volume and close it off even more!
Explain fetal circulation of the heart
Fetal circulation uses right to left shunts
-Fetus gets oxygen from the placenta via the umbilical vein. The oxygenated blood goes to the fetal RA and 2 shunts help it bypass the nonfunctioning lungs
-Foramen ovale (between two atria) shunts 2/3 of blood from RA–> LA –> LV –> aorta to supply head
-Ductus Arteriosus shunts from pulmonary artery to aorta (systemic circulation)
What happens at birth when the baby cries?
Oxygen enters the lungs, pulmonary vascular resistance decreases, and then both shunts begin to close!
What is a patent ductus arteriosus? Why does it happen?
Persistent connection between PA and aorta
Continued prostaglandin (E1) production promotes patency
Who does PDA occur in most times (Risks?)
What are the symptoms of PDA?
Prematurity, Females, Congenital Rubella, Hypoxia
Most asymptomatic. Frequent lower respiratory infections. Labored breathing. Failure to thrive.
Bounding peripheral pulses!!!
What does the murmur of PDA sound like?
Continuous, machine like murmur at LUSB at the pulmonic area
Explain what Eisenmenger Syndrome is and how it is related to PDA
If PDA goes untreated, it can lead to pulmonary HTN and then shunt reversal. So, instead of being a left–> right, it becomes right–> left. The patient becomes cyanotic and clubbing of the feet happens.
The lungs have to pump more and more. The shunt reverses if pulmonary pressure > systemic pressure.
Diagnostics (initial and gold standard) for PDA. What is the first line treatment for PDA?
Echo: initial
Catheterization: gold standard, definitive
NSAIDs (IV Indomethacin) is first line to help close the duct
If no response to Indomethacin, what should be done for the patient with PDA?
Surgery (occlusion)
What is the MC congenital heart disease?
What are the components?
Tetralogy of Fallot
PROVe
–Pulmonary Stenosis –> RV outlet obstruction
–RVH
–Overriding aorta
–VSD
What is one risk factor that should be remembered with ToF?
Chromosome 22 deletion
Symptoms of Tetralogy of Fallot
-Cyanosis (MC symptom)
-Digital Clubbing
-Tet spells: cyanosis relieved with squatting (improves oxygenation)
Explain the murmur of ToF
-Harsh systolic murmur at LUSB (VSD) and right ventricular heave (RVH)
ToF is associated with a number of other conditions. What are they?
Hypospadias, Skeletal malformations, cleft palate
What does a CXR for ToF show?
What is the diagnostic of choice?
Boot shaped heart (large RV)
Echo is DOC
Treatment for ToF
Open ductus arteriosus with prostaglandin E1 to improve circulation
Surgical repair in first 4-12 months of life
What medication should be given to a patient in a current Tet Spell?
BB
MC type of ASD
What occurs in an ASD?
Ostium Secundum
left –> right shunt. The blood is oxygenated multiple times, lots of pulmonary circulation –> pulmonary HTN
Symptoms of ASD
-Exertional dyspnea
-FTT
-HF, syncope
-URIs, poor weight gain
Explain the murmur of an ASD
Systolic ejection crescendo-decrescendo flow murmur at pulmonic area (LUSB)
-Wide, fixed split S2 that doesn’t vary with inspirations
Diagnostics for ASD
-Echo: BEST
-ECG: RBBB
-CXR: Cardiomegaly
-Catheterization: definitive
Treatment for an ASD is based on size, explain.
If < 5mm: observe
If > 1 cm: surgery (between 2-4 years old)
Coarctation of the aorta is narrowing of the aortic lumen. This MC occurs at what location? What does this result in?
-At the location of the ductus arteriosus distal to the left subclavian vein
-Hypertension of the UE and hypotension of the LE
Symptoms of coarctation of the aorta
-Bilateral claudication
-UE hypertension and LE hypotension
-Diminished or delayed LE pulses
-Failure to thrive, poor feeding in infants
-Respiratory distress, grey baby
What are some things Coarctation of the Aorta is associated with?
Turner Syndrome
Bicuspid Aortic Valve
Mitral Valve Defects
Explain the murmur of Coarctation of the aorta
-Systolic murmur radiating to the back, chest, or scapula (due to intercostal muscle collateral arteries developed)
Diagnostics for Coarctation of the Aorta and what they show:
CXR:
Echo:
What is the gold standard?
CXR: Posterior rib notching, 3 sign due to intercostal artery flow
Echo: narrowing of the aorta
Gold standard: angiography
Management for coarctation of the aorta
-Corrective surgery (balloon angioplasty with or without stent)
What should be done BEFORE surgery is pursued?
Give prostaglandin E1 (Alprostadil) to maintain PDA preoperatively
Explain what Transposition of the Great Arteries is (TOGA) and What the MC type is
Where the aorta arises from the RV and the pulmonary artery arises from the LV
Dextro-TGA: two parallel circuits
What happens in the Dextro-TGA type?
Systemic system sends deoxygenated blood back into circulation and the pulmonary system sends oxygenated blood to lungs.
-Survival is dependent on shunts (ASD, VSD, PDA)
Symptoms of ToGA
Cyanosis within first 30 days of life, tachypnea
Diagnostics for ToGA
-CXR: egg on string appearance
-Echo
-Catheter is gold standard, used rarely
Treatment for ToGA
-Arterial switch operation after given Prostaglandin E1 to maintain PDA
MC type of congenital heart disease in children
VSD
What does the murmur of a VSD sound like?
High pitched, harsh holosystolic murmur heard best at LSB
Best diagnostic for VSD
Treatment?
Echo
Observation if asymptomatic
Patch closure if symptomatic
MC innocent (physiologic) murmur?
Still’s Murmur
Explain what a Still Murmur sounds like
Musical, vibratory, twangy, noisy mid-systolic ejection murmur best heart at LLSB and apex
-Minimal to no radiation
What is the MC continuous benign murmur
Cervical Venous Hum
Explain what a cervical venous hum murmur sounds like
Soft, whirling low pitched continuous murmur best heart in RSB in upright position
No radiation
Decreased intensity with rotation/flexion of the head (blood returns to heart at junction of SVC and jugular vein)
