Updates on the indications, timing and outcome of univentricular repair. Updates on the indications, timing and outcome of univentricular repair
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Updates on the indications, timing and outcome of univentricular repair.
A heart that lacks two well-developed ventricles:
Tricuspid atresia+/- pulmonary atresia with intact ventricular septum, double inlet left ventricle, double outlet right ventricle, unbalanced atrioventricular septal defects and hypoplastic left heart syndrome.
Generally, the risk to siblings and offspring of affected individuals is 2% to 5% (19% -33% of siblings HLHS) .
Create unobstructed systemic outflow, unobstructed systemic and pulmonary venous return
Balanced circulation: No early intervention
Advantages of mBTS:
The long-term effects of marked single-ventricle preload and
Modified Norwood 1
Risk factors for midterm mortality were cardiorespiratory failure requiring ventilation and ACS ≥20, but not shunt type.
Hybrid vs NW:
The advantage of the BDG/HemiFontan over
Setting: Cyanosis or pulmonary overcirculation or balanced
Benefits of Cavopulmonary
Operative mortality rate of BDG/HemiFontan is 5%-7%
Modified Fontan operation:
LATERAL TUNNEL FONTAN
STS Congenital Heart Surgery Database: 2,747 Fontan operations (2000 to 2009) in 68 centers
Age above 4 years
Negative prognostic factors for both early and late outcome:
Three groups of PA sizes using the PA index (PAI)
ACC/AHA 2008 GUIDELINES GUCH
Recent 15-year survival reports post-Fontan:
Norwood 1 operation (n=9):100% early mortality rate
Physical and Psychosocial Summary scores of the Child Health Questionnaire Parent Form were within the normal range for over 80% of patients.
Finding of apparently impaired systolic function in the RV subgroup relative to the LV and mixed subgroups is consistent with the general opinion that the structure of the RV is suboptimal for a systemic ventricle.
Arrhythmia occur in 10-40% of patients regardless of the type of Fontan.
Lymphatic circulation may be affected by high venous pressure and impaired thoracic duct drainage.
During a 10-yr follow-up, PLE incidence is about 13%.
Plastic bronchitis : less than 1%–2% of patients.
Circulating ‘‘hepatic factors,’’ which are responsible for either stimulating a repressor of pulmonary arteriovenous malformations (PAVMs) or repressing an activator of PAVMs.
The ventricle evolves from being volume overloaded and overstretched, to overgrown and (severely)underloaded.
Staged vs. Primary Fontan operation (or total cavopulmonary connection)
SVC and IVC to PA connections originally were performed at the same time, which in many patients resulted in a marked increase in blood flow to the lungs, pulmonary lymphatic congestion, and prolonged problems due to pleural effusions.
Better outcome for staged TCPC because of the gradual ventricular unloading and remodelling.
One-stage modified Fontan in low-risk patients (n=15): at least 4 years for the lateral tunnel, and older than 6 years
The 1-stage modified Fontan procedure may put patients at higher surgical risk due to poor cardiovascular adaptation.
The reported impact of prenatal diagnosis on overall survival is variable
All patients see a progressive decline in their exercise capacity, but the decrease in exercise capacity was accelerated if the Fontan surgery was performed in adolescents and adults rather than in children.
Fifteen patients underwent TCPC completion: extracardiac conduit (n = 13), lateral tunnel (n =1), and direct anastomosis of the inferior vena cava and pulmonary trunk (n = 1)
Overall mortality rate was 13.3%.
Long-standing cyanosis and volume overload to the
Only 13% to 16% of patients who underwent Fontan at 3
ESC GUIDELINES ON GUCH 201O
Indications: Small pulmonary arteries
Indications for fenestration closure
After closure of Fontan fenestration: No change in peak exercise capacity as demonstrated by percent of predicted VO2, percent predicted ventilatory anaerobic threshold, heart rate, or O2 pulse.
All patients with clinical thromboembolic events were taking warfarin at the time of the event.
Most individual patients palliated with the Fontan procedure in the 1970s and 1980s have cognitive outcome and academic function within the normal range, but the performance of the cohort is lower than that of the general population.
By 20–28 weeks gestation, myocardial oxygen consumption and heart rate normally increase by 20% and stroke volume by 40%
Women can successfully complete pregnancy after adequate Fontan palliation without important long-term sequelae, although it is often complicated by clinically significant (non-)cardiac events. I
Pregnancy is contraindicated in patients with severely reduced pulmonary blood flow or with severe pulmonary vascular disease (Eisenmenger syndrome) or if ventricular function is poor.
Early staged operation:
The average 80% twenty-year survival rate of Fontan operation is based on a mix of patients who had their procedures done before the advent of and after the TCPC, hence, the outcome of current surgical trends could be better.
In 1971, Fontan and Baudet described an atriopulmonary anastomosis as definitive palliation for tricuspid atresia.
CXR: assess lung disease
All patients with a Fontan circulation have an abnormal cardiorespiratory response to exercise.
Functional health status: Over 80% of subjects scored in the normal range on the CHQ.
Arrhythmia incidence is similar between EC & LT due to:
Due to the absence of pulsatile blood flow and underfilling of the pulmonary vascular bed, patients with Fontan circulation are at increased risk for formation of pulmonary arteriovenous malformations.
Typically, cardiac output in a Fontan circulation at rest is decreased to 70% (range 50–80%) of normal for body surface area.
Decreasing age, as well as intervals, in staged Fontan palliation have beneficial influence on major complications and outcome.
Indications to leave a pulmonary antegrade flow:
Kawashima procedure results in a ‘near complete’ Fontan circulation
Early redirection of hepatic flow after the Kawashima procedure may actually decrease or result in resolution of PAVMs.
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