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The Year in Congenital Heart DiseaseThe Year in Congenital Heart Disease
Perspective: The following are 10 points to remember
about the past year in congenital heart disease:
1. van den Berg et al. studied exercise performance, biventricular
reserve, and N-terminal pro-B-type natriuretic peptide, and exercise
performance in relation to right ventricular (RV) volume in patients
following repair of tetralogy of Fallot (TOF) at less than 2 years of
age. At mid-term follow-up, all parameters were well-preserved, and
unrelated to RV volume (Int J Cardiol 2009;133:363-70).
2. Knauth et al. used cardiac magnetic resonance to predict clinical
outcomes in patients with TOF, with a median time from clinical repair
to evaluation of 21 years. Adverse outcomes, defined as death, sustained
ventricular tachycardia (VT), and increase in New York Heart
Association functional class to grade II or IV, occurred in 21% of
patients. Severe RV dilation and right or left ventricular systolic
dysfunction predicted major adverse events (Heart
2008;94:211-6).
3. In an additional study of pulmonary insufficiency after tetralogy
repair, Harrild and colleagues compared patients with TOF and RV
dilatation who had undergone pulmonary valve replacement, as compared
with those who had not. No significant differences were seen in sudden
death, VT, or a combined endpoint of sudden death/VT in patients who had
undergone pulmonary valve replacement (Circulation
2009;119:445-51).
4. Shiraishi et al. studied the impact of the age at Fontan on exercise
capacity, hemodynamics, and ventricular function in children with
systemic left ventricles. VO2max, left ventricular ejection
fraction, and cardiac index were all higher in patients undergoing
Fontan at <3 years of age (Ann Thorac Surg 2009;87:555-61).
5. The Nationwide Inpatient Sample was used by Karamlou and colleagues
to determine surgical practice patterns for adults with congenital heart
disease. In-hospital mortality was lower (1.87%) for adults operated on
by pediatric heart surgeons, as compared with 4.84% for those operated
on by nonpediatric heart surgeons (Circulation
2008;118:2345-52).
6. Vida et al. studied the optimal timing of patent ductus arteriosus
ligation in premature infants. Medical treatment was successful in 149
of 201 patients. More than two cycles of ibuprofen were associated with
increased risk for bronchopulmonary dysplasia and acute renal failure (Ann
Thorac Surg 2009;87:1509-16).
7. Bové et al. investigated 93 children following the arterial switch
procedure for D-transposition of the great arteries (D-TGA). At a mean
follow-up of 5 years, aortic regurgitation >2+ developed in 10% of
patients with D-TGA with intact ventricular septum and 23% of patients
with D-TGA and ventricular septal defect (VSD). Freedom from
re-intervention at 1, 5, and 10 years was 98%, 96%, and 96% for
D-TGA/intact VSD and 65%, 63%, and 63% for D-TGA/VSD (Ann Thorac
Surg 2008;85:823-30).
8. Because of a previous study implicating balloon atrial septostomy
(BAS) as a cause of brain injury, Petit and colleagues reported on
magnetic resonance imaging of patients prior to surgical repair of TGA.
Of 26 patients, 14 underwent BAS. No strokes occurred, although 10 of 26
patients were found to have hypoxic brain injury in the form of
periventricular leukomalacia. No association was seen between this
finding and BAS. Infants with periventricular leukomalacia had lower
preoperative oxygenation and longer time to surgery than infants without
the finding (Circulation 2009;119:709-16).
9. Sharma et al. reported outcomes of children undergoing anatomic
repairs for congenitally corrected TGA. A total of 31 patients underwent
an atrial switch/Rastelli procedure with 17% early deaths and no late
deaths. A total of 37 patients underwent a ‘double switch’ with 13.5%
early mortality and 10.8% late mortality. An additional four patients
required re-operations, four had left ventricular ejection fraction
<40%, five had moderate aortic regurgitation, and five patients had
symptomatic tricuspid insufficiency, one with tricuspid stenosis, and
one with superior vena cava obstruction (J Thorac Cardiovasc Surg
2009;137:404-12).
10. Tabbutt et al. studied neurodevelopmental outcomes for 83 infants
undergoing staged palliation for hypoplastic left heart syndrome at 1
year of age. The neuromuscular examination was abnormal or suspect in
65% of patients. The mean Mental Developmental Index score was 90, and
20 patients had scores <70. The mean Psychomotor Development Index
was 74, with 42 patients having scores <70. In multivariate analysis,
younger gestational age, presence of a genetic syndrome, and need for
preoperative intubation had negative effects on neurodevelopmental
outcomes. Operative factors, including duration of deep hypothermic
circulatory arrest, were not shown to impact neurodevelopmental outcomes
(Pediatrics 2008;121:476-83).
Full text at JACC
about the past year in congenital heart disease:
1. van den Berg et al. studied exercise performance, biventricular
reserve, and N-terminal pro-B-type natriuretic peptide, and exercise
performance in relation to right ventricular (RV) volume in patients
following repair of tetralogy of Fallot (TOF) at less than 2 years of
age. At mid-term follow-up, all parameters were well-preserved, and
unrelated to RV volume (Int J Cardiol 2009;133:363-70).
2. Knauth et al. used cardiac magnetic resonance to predict clinical
outcomes in patients with TOF, with a median time from clinical repair
to evaluation of 21 years. Adverse outcomes, defined as death, sustained
ventricular tachycardia (VT), and increase in New York Heart
Association functional class to grade II or IV, occurred in 21% of
patients. Severe RV dilation and right or left ventricular systolic
dysfunction predicted major adverse events (Heart
2008;94:211-6).
3. In an additional study of pulmonary insufficiency after tetralogy
repair, Harrild and colleagues compared patients with TOF and RV
dilatation who had undergone pulmonary valve replacement, as compared
with those who had not. No significant differences were seen in sudden
death, VT, or a combined endpoint of sudden death/VT in patients who had
undergone pulmonary valve replacement (Circulation
2009;119:445-51).
4. Shiraishi et al. studied the impact of the age at Fontan on exercise
capacity, hemodynamics, and ventricular function in children with
systemic left ventricles. VO2max, left ventricular ejection
fraction, and cardiac index were all higher in patients undergoing
Fontan at <3 years of age (Ann Thorac Surg 2009;87:555-61).
5. The Nationwide Inpatient Sample was used by Karamlou and colleagues
to determine surgical practice patterns for adults with congenital heart
disease. In-hospital mortality was lower (1.87%) for adults operated on
by pediatric heart surgeons, as compared with 4.84% for those operated
on by nonpediatric heart surgeons (Circulation
2008;118:2345-52).
6. Vida et al. studied the optimal timing of patent ductus arteriosus
ligation in premature infants. Medical treatment was successful in 149
of 201 patients. More than two cycles of ibuprofen were associated with
increased risk for bronchopulmonary dysplasia and acute renal failure (Ann
Thorac Surg 2009;87:1509-16).
7. Bové et al. investigated 93 children following the arterial switch
procedure for D-transposition of the great arteries (D-TGA). At a mean
follow-up of 5 years, aortic regurgitation >2+ developed in 10% of
patients with D-TGA with intact ventricular septum and 23% of patients
with D-TGA and ventricular septal defect (VSD). Freedom from
re-intervention at 1, 5, and 10 years was 98%, 96%, and 96% for
D-TGA/intact VSD and 65%, 63%, and 63% for D-TGA/VSD (Ann Thorac
Surg 2008;85:823-30).
8. Because of a previous study implicating balloon atrial septostomy
(BAS) as a cause of brain injury, Petit and colleagues reported on
magnetic resonance imaging of patients prior to surgical repair of TGA.
Of 26 patients, 14 underwent BAS. No strokes occurred, although 10 of 26
patients were found to have hypoxic brain injury in the form of
periventricular leukomalacia. No association was seen between this
finding and BAS. Infants with periventricular leukomalacia had lower
preoperative oxygenation and longer time to surgery than infants without
the finding (Circulation 2009;119:709-16).
9. Sharma et al. reported outcomes of children undergoing anatomic
repairs for congenitally corrected TGA. A total of 31 patients underwent
an atrial switch/Rastelli procedure with 17% early deaths and no late
deaths. A total of 37 patients underwent a ‘double switch’ with 13.5%
early mortality and 10.8% late mortality. An additional four patients
required re-operations, four had left ventricular ejection fraction
<40%, five had moderate aortic regurgitation, and five patients had
symptomatic tricuspid insufficiency, one with tricuspid stenosis, and
one with superior vena cava obstruction (J Thorac Cardiovasc Surg
2009;137:404-12).
10. Tabbutt et al. studied neurodevelopmental outcomes for 83 infants
undergoing staged palliation for hypoplastic left heart syndrome at 1
year of age. The neuromuscular examination was abnormal or suspect in
65% of patients. The mean Mental Developmental Index score was 90, and
20 patients had scores <70. The mean Psychomotor Development Index
was 74, with 42 patients having scores <70. In multivariate analysis,
younger gestational age, presence of a genetic syndrome, and need for
preoperative intubation had negative effects on neurodevelopmental
outcomes. Operative factors, including duration of deep hypothermic
circulatory arrest, were not shown to impact neurodevelopmental outcomes
(Pediatrics 2008;121:476-83).
Full text at JACC
