Pulmonary Artery Dilatation Due to Pressure or Volume Overload in Congenital Heart Disease.

Background: Pulmonary artery dilatation is described mostly in association with pulmonary hypertension. Patients/Methods: Study analysis: 60 patients with pulmonary arterial hypertension in congenital heart disease (PAH-CHD); 64 with repaired tetralogy of Fallot/pulmonary regurgitation (rTOF/PR); and 80 healthy (NORMAL). Measured were: main pulmonary artery (MPA) diameter and MPA/ascending aorta (Ao asc) ratio, by echocardiography (ECHO) and computer tomography or magnetic resonance imaging (CT/MRI). Results: In MPA diameter, significant differences between PAH-CHD, rTOF/PR, and NORMAL were found (median): 37 vs. 27 vs. 21 mm (p < 0.0001). In MPA/Ao asc ratio, there was a difference between PAH-CHD and NORMAL (median): 1.3 vs. 0.8 (p < 0.0001), but not between rTOF/PR and NORMAL: 0.74 vs. 0.8 (p = 0.3). Significant MPA dilatation (>40 mm) was present: in PAH-CHD, 35% (ECHO) and 76.9% (CT/MRI) of patients, while in rTOF/PR, 3.1% (ECHO) and 7.8% (CT/MRI). Severe MPA dilatation (>50 mm) occurred only in PAH-CHD: 16.7% (ECHO) and 31.4% (CT/MRI), while not in rTOF/PR. There was a significant correlation between ECHO and CT/MRI measurements, but ECHO was underestimated in all parameters. Conclusions: MPA dilatation due to pressure overload is more frequent and more severe; volume overload also leads to MPA dilatation but is less severe. The MPA/Ao asc ratio is not reliable for MPA dilatation estimation in rTOF/PR.

Immune Abnormalities in Patients With Single Ventricle Circulation Precede the Fontan Procedure.

BACKGROUND: Immune abnormalities are common in Fontan patients with protein-losing enteropathy. Limited data exist on immune function of other patients with single ventricle circulation. METHODS: This prospective cohort study evaluated immunologic characteristics of children with single ventricle circulation from neonatal age up to early post-Fontan period. RESULTS: Low leukocyte counts were observed in half of the patients prior to bidirectional Glenn and Fontan surgery. Total lymphocyte counts were below normal range in 36% to 63% of patients across all groups except patients following Fontan procedure who had normal counts. Typical lymphocyte subpopulation patterns were (1) high counts of total and helper T lymphocytes (CD3+ and CD4+ cells), low B lymphocytes (CD19+ cells), and increased CD4/CD8 ratio in neonates and (2) low T lymphocytes (CD3+, CD4+, CD8+ cells) with high natural killer cells (CD16+) and B lymphocytes (CD19+ cells) in other groups. Low preoperative total lymphocyte counts were associated with longer intensive care unit stay in patients after bidirectional Glenn and Fontan procedure ( P = .03 and P = .01, respectively) and low leukocyte counts with higher incidence of pleural effusions and chylothorax after Fontan procedure ( P = .005 and P = .002, respectively). CONCLUSIONS: Single ventricle patients display several immunological abnormalities. Beyond the neonatal age, an immune pattern includes CD3+, CD4+, CD8+ lymphopenia, and CD16+ and CD19+ lymphocytosis. B-cell lymphocytosis compensates T-cell lymphopenia, producing normal total lymphocyte counts in patients early after Fontan surgery. Low preoperative total lymphocyte counts may be associated with longer postoperative intensive care unit stay in patients with bidirectional Glenn and Fontan procedure and leukopenia with pleural effusions in Fontan patients.

Natural history of newborn with borderline small left heart without interatrial communication.

A patient with a borderline small left heart requires difficult clinical decision making during prenatal and neonatal periods. This report describes the unusual natural history of a newborn with prenatally diagnosed hypoplastic left heart structures and early spontaneous postnatal closure of the interatrial communication. Initial deterioration from pulmonary edema and low-cardiac output improved with postnatal intensive care. During the seventh week of life, aortic coarctation was diagnosed and the infant underwent uneventful surgical repair. Despite transient clinical deterioration, early spontaneous closure of the interatrial communication in a newborn with borderline small left heart could be associated with gradual hemodynamic adaptation to postnatal biventricular circulation.

Initial experience with polytetrafluoroethylene leaflet extensions for aortic valve repair.

OBJECTIVES: The purpose of this study is to evaluate our initial experience with aortic valve repair using polytetraflouroethylene (PTFE) leaflet extensions in congenital valvular disease. METHODS: From October 2008 through February 2011, 13 patients underwent aortic valvuloplasty by PTFE leaflet extensions. All valves were repaired in a tri-leaflet configuration using PTFE leaflet extensions. The median age at operation was 14 years (1.8-19.7 years) and the median weight was 58 kg (9.5-86 kg). Previous interventions included balloon valvuloplasty in two patients, aortic valvuloplasty in one and coarctation repair in one patient. Eight (73%) patients had combined aortic stenosis and insufficiency, three (23%) had isolated insufficiency and two (15%) had stenosis only. In 10 (77%) patients, a bicuspid aortic valve was present. RESULTS: The follow-up ranged from 2 to 30 months (mean follow-up 14.8 ± 9 months). At the latest echocardiography follow-up, six patients had none or trace aortic insufficiency, six patients had a mild aortic insufficiency and one patient had a mild-to-moderate insufficiency. The mean aortic insufficiency degree decreased from 1.8 ± 1.2 preoperatively to 0.8 ± 0.6 at the follow-up (P < 0.01). The mean gradient across the aortic valve decreased from 56 ± 40 mmHg preoperatively to 12 ± 13 mmHg at the follow-up (P < 0.0008). All patients are alive. There were no reoperations. The median hospital stay was 9 days (4-21 days). CONCLUSIONS: The use of PTFE leaflet extensions is an effective technique for aortic valve reconstruction in congenital valvular disease. Long-term follow-up is necessary to assess the durability of this type of repair.

Ross-konno procedure in children: midterm results.

The aim of this study was to analyze the midterm results following the Ross-Konno procedure in children. Between 1999 and 2008, 29 patients with complex left ventricular outflow tract obstruction underwent the Ross-Konno procedure. There were 12 (41%) infants (group A) and 17 (59%) older patients (group B). The median age at operation was 3.3 years (range, 6 days to 16 years). At 7 years of follow-up, survival was 96% (1 late death), with no differences between groups A and B. Freedom from aortic regurgitation ≥ mild was 81%, with no differences between groups A and B. No residual gradient was noted in the left ventricular outflow tract in either group. Freedom from mitral regurgitation ≥ mild was 100% in group B and 41% in group A (P = .0029). The mitral regurgitation was associated with morphological abnormalities of the mitral valve and with development of endocardial fibroelastosis after failed intervention during the newborn period. Freedom from reoperation was 73% in group B and 24% in group A (P = .0093). All patients are now in sinus rhythm, and 43% are without medication. With the technical aspects of this procedure well accomplished, mortality is low, and the functional outcome is encouraging. At midterm follow-up, there was no residual or recurrent outflow tract obstruction and an excellent function of the neoaortic valve. The higher incidence of mitral regurgitation in infants, which was associated with morphological abnormalities of mitral valve and development of endocardial fibroelastosis, is worrisome.

Primary early correction of tetralogy of Fallot irrespective of age.

OBJECTIVE: The policy of early repair of patients with tetralogy of Fallot, irrespective of age, as opposed to initial palliation with a shunt, remains controversial. The aim of our study was to analyze the midterm outcome of primary early correction of tetralogy of Fallot. METHODS: Between 1996 and 2005, a total of 61 consecutive patients less than 6 months of age underwent primary correction of tetralogy of Fallot in two institutions. The median age at surgery was 3.3 months, and 27 patients (44%) were younger than 3 months of age, including 12 (20%) newborns. We analyzed the patients in 2 groups: those younger than 3 months of age, and those aged between 3 and 6 months. RESULTS: There was one early (1.6%), and one late death. Actuarial survival was 98.4%, 96.7%, 96.7% at 1, 5, and 10 years respectively, with a median follow up of 4.5 years. There was no difference in survival, bypass time, lengths of ventilation, and hospital stay between the groups. A transjunctional patch was placed significantly more often in the patients younger than 3 months (p = 0.039), with no adverse effect on survival and morbidity during the follow-up. Freedom from reoperation was 98.2%, 92.2%, and 83% at 1, 5, and 10 years respectively, with no difference between the groups. CONCLUSION: Elective primary repair of tetralogy of Fallot in asymptomatic patients is delayed beyond 3 months of age. In symptomatic patients, primary repair of tetralogy of Fallot is performed irrespective of age, weight and preoperative state. This approach is safe, and provides an excellent midterm outcome with acceptable morbidity and rates of reintervention. The long-term benefits of this approach must be established by careful follow-up, with particular emphasis on arrhythmias, right ventricular function, and exercise performance.