Adult Congenital Heart Disease Flashcards
What is the typical presentation of ASDs in adults?
Shortness of breath or palpitations, often discovered incidentally
ASDs can also be found during workup for stroke due to paradoxical embolization.
What is a ‘positive’ step-up in oxygen saturation at the atrial level?
≥7% (absolute difference in mean saturation values)
A ‘positive’ step-up at ventricular or pulmonary arterial levels must be ≥5%.
What method is used to calculate pulmonary blood flow assuming a specific oxygen consumption?
The Fick method
Assumes oxygen consumption of the lungs to be 125 mL/min/m².
Which systemic venous saturation is usually higher, IVC or SVC?
IVC saturation is typically higher than SVC saturation
This is because the kidney receives 25% of the cardiac output and consumes less oxygen.
What formula is used to estimate mixed venous saturation?
The Flamm formula
This formula is used in situations where direct measurements are not available.
When is ASD closure indicated according to recent guidelines?
Evidence of RA or RV enlargement, paradoxical embolism, or platypnea–orthodeoxia
Closure may also be considered for net left-to-right shunting with pulmonary arterial pressure <2/3 systemic pressure.
What is the significance of a QP:QS ratio of >1.5:1?
It has traditionally been used to define a shunt that should be closed
This definition may not be the best for adults with long-standing right ventricular volume overload.
What is a common complication associated with pulmonary arteriovenous malformations?
Stroke, brain abscess, life-threatening hemoptysis or hemothorax, and hypoxemic respiratory failure
Pulmonary arteriovenous malformations can cause serious complications and are often congenital.
What should be considered if dilation of the coronary sinus is observed?
The possibility of a persistent left-sided vena cava
This is typically normal physiology with no clinical manifestations.
What are the signs and symptoms supporting a diagnosis of PDA?
Dyspnea on exertion, widened pulse pressure, left subclavicular murmur
The murmur is classically described as a continuous ‘machinery’ murmur.
What is Eisenmenger’s syndrome?
A condition where pulmonary arterial pressure exceeds systemic pressure due to chronic left-to-right shunting
It leads to a reversal of shunt flow and consequent cyanosis.
What is the recommended follow-up for patients with small PDA and no left heart volume overload?
Clinical follow-up every 3 to 5 years
Endocarditis prophylaxis is not recommended for asymptomatic patients with unrepaired PDA.
What is the ideal timing for surgical correction of defects resulting in significant pulmonary overcirculation?
Before 2 years of life for VSDs and before 6 months for AV canal defects and conotruncal abnormalities
Early correction is critical to prevent irreversible pulmonary vascular disease.
What is the prognosis for patients with Eisenmenger’s syndrome compared to those with idiopathic pulmonary arterial hypertension?
77% survival vs. 35% survival at 3 years, untreated
Despite a better prognosis, it remains a serious condition.
What is the gold standard of treatment for pulmonary arteriovenous malformations?
Embolotherapy
This is the preferred method when possible to manage these malformations.
What does the Fick method calculate in the context of cardiac shunts?
Pulmonary blood flow, systemic blood flow, and shunt flow
It is essential for determining the hemodynamics of congenital heart defects.
What is heart-lung transplant used for?
End-stage Eisenmenger’s syndrome treatment
Eisenmenger’s syndrome is a complication of congenital heart disease that leads to pulmonary vascular disease.
What is the risk of late surgical correction with an arterial switch procedure after pulmonary vascular disease has developed?
Significant mortality risk
Does pulmonary artery banding alter the disease course in pulmonary vascular disease?
No
How does administration of 100% oxygen affect systemic oxygen saturation in patients with right-to-left shunting?
Will not significantly improve
What has long-term oxygen therapy in Eisenmenger’s syndrome been shown to improve?
Nothing (symptoms, functional capacity, or survival)
Sandoval J, et al. Am J Respir Crit Care Med 2001;164(9):1682–1687.
What is the most common congenital anomaly of the tricuspid valve?
Ebstein anomaly
In Ebstein anomaly, which leaflet of the tricuspid valve arises from the normal position?
Anterior leaflet
What characterizes the right ventricle in Ebstein anomaly?
Divided into two chambers: atrialized RV and functional RV of variable size
What is a common communication present in Ebstein anomaly?
Atrial level communication (PFO or ASD)
What mechanisms promote atrial level right-to-left shunting in Ebstein anomaly?
- Right ventricular outflow tract obstruction
- Poor RV filling
- Tricuspid valve regurgitation
- RV failure
- Increased pulmonary vascular resistance
How do patients with right-to-left shunting in Ebstein anomaly experience systemic venous congestion compared to those with an intact septum?
Do not easily develop systemic venous congestion
What is the management approach for asymptomatic patients with Ebstein anomaly?
Periodic monitoring only
What may patients with exertional cyanosis secondary to atrial level shunting benefit from?
Percutaneous device closure
What does temporary balloon occlusion of a defect in Ebstein anomaly lead to?
Elevation of RA pressure and a drop in cardiac output
What are common surgical interventions for severe symptoms in Ebstein anomaly?
- Tricuspid valve repair or replacement
- Shunt closure
- Ventricular plication
- Atrial reduction
- Antiarrhythmic surgery
What recent evidence suggests can improve the prognosis of surgical management in Ebstein anomaly?
Aggressive preoperative treatment
What characterizes Tetralogy of Fallot (TOF)?
- Pulmonic stenosis
- VSD
- Right ventricular hypertrophy
- Overriding aortic valve
What was historically done prior to performing a complete repair of TOF?
Palliative Blalock–Taussig shunts were employed
What is a common late complication after surgical repair of TOF?
Pulmonary insufficiency
What is a frequent complication encountered in adults with TOF, especially those with pulmonary atresia?
Formation of aortopulmonary collaterals
What is the typical origin of coronary artery fistulae?
Persistence of sinusoids from early embryogenesis
What percentage of coronary fistulae originate from the RCA?
55%
What can coronary fistulae lead to besides being small and usually asymptomatic?
Myocardial ischemia distal to the fistula
What is transposition of the great arteries characterized by?
Two separate circulations requiring a shunt for infantile survival
What is a common complication after the Sening and Mustard procedures for transposition of the great arteries?
Failure of the systemic (right) ventricle
What is Scimitar syndrome characterized by?
- Hypoplasia of the right lung
- Anomalous right pulmonary venous drainage to the IVC
- Aortopulmonary collateral flow
What is the most common congenital coronary anomaly?
Origin of the LCx from the RCA
What is the peak-to-peak pressure gradient mentioned in the text?
51 mm Hg
What factors contribute to the pathophysiology of hypertension in patients with coarctation of the aorta?
- Activation of the renin–angiotensin–aldosterone system
- Decreased renal blood flow
What is the survival rate of patients with unoperated coarctation of the aorta?
35 years
What is a common indication for balloon angioplasty and/or stent placement in coarctation patients?
Gradient of 20 mm Hg or more
What is depicted in Figure A32-18?
A right anterior oblique (RAO) angiographic image of an anomalous left main coronary artery originating from the right coronary cusp
What is the association of the anomalous left main coronary artery?
Increased risk of sudden death in children and young adults
What is the peak-to-peak pressure gradient threshold for valvuloplasty in asymptomatic patients with a doming pulmonic valve?
> 40 mm Hg
What is the recommended management for asymptomatic patients with a peak-to-peak gradient <30 mm Hg?
Safe observation
What are the surgical indications for patients with valvular pulmonic stenosis?
- Right ventricular systolic pressure >80 mm Hg
- Dysplastic valve morphology
- Severe pulmonic stenosis with severe pulmonary regurgitation
- Associated hypoplastic pulmonary anulus
What is the so-called suicide RV?
Stenosis at the level of the valve is relieved, causing dynamic obstruction along the infundibulum when right ventricular pressures exceed 100 mm Hg
What is the estimated death rate during pulmonary balloon valvuloplasty?
0.24%
What are some acute complications of pulmonary balloon valvuloplasty?
- Ventricular ectopy
- AV node block
- Pulmonary regurgitation
What anatomical and functional abnormalities are associated with the heart after repair of Tetralogy of Fallot (TOF)?
- Pulmonary valve insufficiency
- Conduction delay (right bundle-branch block)
- Right ventricular dilation
- Increased pulmonary insufficiency
- Ventricular dysfunction
What is the median lifespan of a bioprosthetic valve?
10 years
What are the indications for valve replacement in patients with repaired TOF?
- Symptoms of exercise intolerance or heart failure
- QRS duration >180 ms
- QRS prolongation >3.5 ms/year
- Sustained arrhythmia
- Right ventricular end-systolic volume >85 mL/m2
- Right ventricular end-diastolic volume >170 mL/m2
What characterizes single ventricle physiology?
One functional ventricle must generate sufficient cardiac output for both systemic and pulmonary circulations
What is the purpose of a Glenn procedure?
To route superior venous circulation to the pulmonary arterial system
What are venovenous collaterals and their risk?
Collaterals that feed blood to pulmonary veins, bypassing the lungs, and carry a risk for paradoxical embolization
What is the recommended technique for evaluating subaortic stenosis?
Echocardiography, particularly transesophageal
What is the peak instantaneous gradient that indicates surgical intervention for subaortic stenosis?
50 mm Hg
Who should receive prophylaxis for subacute bacterial endocarditis (SBE)?
- Patients with implanted prosthetic materials
- History of infective endocarditis
- Unrepaired cyanotic congenital heart disease
- Repaired congenital heart disease with residual defects
- Completely repaired congenital heart disease with prosthetic material for 6 months following the procedure
What are the indications for intervention in pulmonary artery stenosis?
- Right ventricular systolic pressure elevation (at least 50% of systemic pressure)
- Translesion gradient of at least 30 mm Hg
- Flow discrepancy to the distal pulmonary vascular bed
- At least 50% luminal narrowing
