Neonatal Outcomes of Critical Congenital Heart Defects: A Multicenter Epidemiological Study of Turkish Neonatal Society : Neonatal Outcomes of CCHD.

Critical congenital heart disease (CCHD) is one of the leading causes of neonatal and infant mortality. We aimed to elucidate the epidemiology, spectrum, and outcome of neonatal CCHD in Türkiye. This was a multicenter epidemiological study of neonates with CCHD conducted from October 2021 to November 2022 at national tertiary health centers. Data from 488 neonatal CCHD patients from nine centers were entered into the Trials-Network online registry system during the study period. Transposition of great arteria was the most common neonatal CHD, accounting for 19.5% of all cases. Sixty-three (12.9%) patients had extra-cardiac congenital anomalies. A total of 325 patients underwent cardiac surgery. Aortic arch repair (29.5%), arterial switch (25.5%), and modified Blalock-Taussig shunt (13.2%). Overall, in-hospital mortality was 20.1% with postoperative mortality of 19.6%. Multivariate analysis showed that the need of prostaglandin E1 before intervention, higher VIS (> 17.5), the presence of major postoperative complications, and the need for early postoperative extracorporeal membrane oxygenation were the main risk factors for mortality. The mortality rate of CCHD in our country remains high, although it varies by health center. Further research needs to be conducted to determine long-term outcomes for this vulnerable population.

Comparison of serum N-terminal pro-brain natriuretic peptide levels, conventional echocardiography, exercise parameters, and dyssynchrony measurements in Fontan patients.

INTRODUCTION: Little data are available concerning the methods used in the long-term follow-up of Fontan patients. We analyzed the association between serum N-terminal pro-brain natriuretic peptide levels, conventional echocardiography findings, exercise parameters, and dyssynchrony measurements in patients who underwent Fontan surgery. METHODS: This study included 28 patients who underwent Fontan surgery (mean age 12.8 ± 4.36 years) and 27 healthy controls (mean age 12.5 ± 3.76 years). Echocardiography examinations and exercise tests were performed in both groups. The systemic ventricle was examined via echocardiography, dyssynchrony measurement was performed, the systemic ventricular myocardial performance index was calculated, and serum N-terminal pro-brain natriuretic peptide levels were measured for all subjects. RESULTS: Lower cardiac output, stroke volume, maximal work, chronotropic index, maximal oxygen uptake, and higher N-terminal pro-brain natriuretic peptide levels were observed in the Fontan group than in the control group (p < 0.05). A negative correlation was found between physical exercise parameters and N-terminal pro-brain natriuretic peptide levels and dyssynchrony measurements. CONCLUSION: Measurements of exercise capacity, serum N-terminal pro-brain natriuretic peptide levels, and dyssynchrony measurement were more valuable than conventional methods for assessing patients' clinical and functional status. Dyssynchrony measurements provided better information about ventricular status than did conventional echocardiography studies. While patients' systolic function determined by conventional echocardiography was normal, dyssynchrony measurements showed the opposite result. The negative relationship between serum N-terminal pro-brain natriuretic peptide levels, dyssynchrony measurements, and exercise capacity suggests that these parameters should be investigated further in Fontan patients.

Results of balloon and surgical valvuloplasty in congenital aortic valve stenosis: A 19-year, single-center, retrospective study.

BACKGROUND: This study aims to compare the success, complications, and long-term outcomes of aortic balloon valvuloplasty and surgical aortic valvuloplasty in pediatric patients with congenital aortic valve stenosis. METHODS: Between March 2000 and October 2019, a total of 267 procedures, including 238 balloon valvuloplasties and 29 surgical valvuloplasties, in 198 children (135 males, 63 females; mean age: 57.4±62.6 months; range, 0.03 to 219 months) were retrospectively analyzed. The hospital records, echocardiographic images, catheterization data, angiography images, and operative data were reviewed. RESULTS: Aortic regurgitation was mild in 73 patients before balloon valvuloplasty, and none of the patients had moderate-to-severe aortic regurgitation. Compared to surgical valvuloplasty, the rate of increase in the aortic regurgitation after balloon valvuloplasty was significantly higher (p=0.012). The patients who underwent balloon valvuloplasty did not need reintervention for a mean period of 46±45.6 months, whereas this period was significantly longer in those who underwent surgical valvuloplasty (mean 80.5±53.9 months) (p=0.018). The overall failure rate was 8%. Moderate-to-severe aortic regurgitation was the most important complication developing due to balloon valvuloplasty in the early period (13%). All surgical valvuloplasties were successful. The mean length of hospitalization after balloon valvuloplasty was significantly shorter than surgical valvuloplasty (p=0.026). During follow-up, a total of 168 patients continued their follow-up, and a reinterventional or surgical intervention was not needed in 78 patients (47%). CONCLUSION: Aortic balloon valvuloplasty can be repeated safely and helps to eliminate aortic valve stenosis without needing sternotomy. Surgical valvuloplasty can be successfully performed in patients in whom the expected benefit from aortic balloon valvuloplasty is not achieved.

Infective endocarditis in childhood: a single-center experience of 18 years

Abstract Introduction: We aimed to present the risk factors, clinical and laboratory findings, treatment management, and risk factors for morbidity and mortality of infective endocarditis (IE) as well as to relate experiences at our center. Method: We retrospectively analyzed data of 47 episodes in 45 patients diagnosed with definite/possible IE according to the modified Duke criteria between May 2000 and March 2018. Results: The mean age of all patients at the time of diagnosis was 7.6±4.7 years (range: 2.4 months to 16 years). The most common symptoms and findings were fever (89.3%), leukocytosis (80.8%), splenomegaly (70.2%), and a new heart murmur or changing of pre-existing murmur (68%). Streptococcus viridans (19.1%), Staphylococcus aureus (14.8%), and coagulase-negative Staphylococci (10.6%) were the most commonly isolated agents. IE-related complications developed in 27.6% of the patients and the mortality rate was 14.8%. Conclusion: We found that congenital heart disease remains a significant risk factor for IE. The highest risk groups included operated patients who had conduits in the pulmonary position and unoperated patients with a large ventricular septal defect. Surgical intervention was required in most of the patients. Mortality rate was high, especially in patients infected with S. aureus, although the time between the onset of the first symptom and diagnosis was short. Patients with fever and a high risk of IE should be carefully examined for IE, and evaluation in favor of IE until proven otherwise will be more accurate. In high-risk patients with prolonged fever, IE should be considered in the differential diagnosis.

Infective Endocarditis in Childhood: a Single-Center Experience of 18 Years.

INTRODUCTION: We aimed to present the risk factors, clinical and laboratory findings, treatment management, and risk factors for morbidity and mortality of infective endocarditis (IE) as well as to relate experiences at our center. METHOD: We retrospectively analyzed data of 47 episodes in 45 patients diagnosed with definite/possible IE according to the modified Duke criteria between May 2000 and March 2018. RESULTS: The mean age of all patients at the time of diagnosis was 7.6±4.7 years (range: 2.4 months to 16 years). The most common symptoms and findings were fever (89.3%), leukocytosis (80.8%), splenomegaly (70.2%), and a new heart murmur or changing of pre-existing murmur (68%). Streptococcus viridans (19.1%), Staphylococcus aureus (14.8%), and coagulase-negative Staphylococci (10.6%) were the most commonly isolated agents. IE-related complications developed in 27.6% of the patients and the mortality rate was 14.8%. CONCLUSION: We found that congenital heart disease remains a significant risk factor for IE. The highest risk groups included operated patients who had conduits in the pulmonary position and unoperated patients with a large ventricular septal defect. Surgical intervention was required in most of the patients. Mortality rate was high, especially in patients infected with S. aureus, although the time between the onset of the first symptom and diagnosis was short. Patients with fever and a high risk of IE should be carefully examined for IE, and evaluation in favor of IE until proven otherwise will be more accurate. In high-risk patients with prolonged fever, IE should be considered in the differential diagnosis.

Diagnosis and treatment of abnormal left coronary artery originating from the pulmonary artery: A single-center experience.

OBJECTIVE: We aimed to review symptoms, findings, surgical treatment options, short- and mid-term outcomes, and reoperation rate of patients diagnosed with of left coronary artery from the pulmonary artery (ALCAPA) of an anomalous origin in our institution. METHODS: From May 2000 to March 2018, 33 patients who had left coronary artery originating from the pulmonary artery were retrospectively examined. The clinical features of patients, diagnostic tools and their efficacy, outcomes of surgical repair, and problems during follow-up were evaluated. RESULTS: Thirty-three patients (22 females, 11 males) were included in the study. At the time of surgery, the median age and weight of patients were 6 months (minimum/maximum, 1-166 months) and 6.5 kg (minimum/maximum, 3-38.5 kg), respectively. The mean follow-up was 5±3.5 years (range, 1-16 years). Dyspnea, tachypnea, diaphoresis, prolonged feeding time, and developmental delay were common presenting signs and symptoms. It was determined that all the patients who were diagnosed at another center reached our center for surgical treatment within 1 month. Twenty-three (69.7%) patients had pathologic Q wave with anterior and/or anterolateral myocardial infarction signs on an electrocardiogram (ECG), whereas 22 (66.6%) patients had ST-T segment changes. Twenty-one (63.6%) patients had cardiomegaly on the telecardiogram. A reimplantation surgery was performed to 22 patients and 10 patients underwent the Takeuchi procedure. In addition to ALCAPA repair, 5 patients needed mitral valve plasty. Atrial septal defect (ASD) and ventricular septal defect (VSD) were closed in one patient, and Tetralogy of Fallot was totally corrected in another. At discharge, there was a significant improvement in left ventricular (LV) systolic functions. At the last visit, all patients had normal LV systolic functions except four who had mild dysfunction. The mean follow-up of the four patients was 2.8 years. In the early postoperative period, complications were seen in 10 patients. Five patients died in the early postoperative period, while one patient died 9 months after the ALCAPA surgery because of low cardiac output syndrome that developed after mitral repair. CONCLUSION: Patients with ALCAPA commonly present with congestive heart failure symptoms. When the diagnosis is confirmed in these patients, surgical treatment should not be delayed. The availability of surgical center and surgery outcomes for ALCAPA diagnosed patients are comparable with other countries, but the delay in the diagnosis of disease is still a problem in our country.

Reversible cardiomyopathy-tachycardiomyopathy in children.

Arslan A, Erdogan I, Varan B, Yilmaz M, Özin MB, Tokel NK. Reversible cardiomyopathy-tachycardiomyopathy in children. Turk J Pediatr 2019; 61: 552-559. Tachycardia-induced cardiomyopathy (tachycardiomyopathy) is defined by the presence of a sustained tachycardia that results in left ventricular systolic dysfunction. Restoration of cardiac function is dependent on the control of tachyarrhythmias. We report a series including ten children with tachycardia-induced cardiomyopathy with different etiologies. The medical records of patients with tachycardiomyopathy who were managed in a Pediatric Cardiology Clinic between the years of 2014-2017 were reviewed retrospectively. Ten children (3 female, 7 male) were diagnosed with tachycardiomyopathy. The median age of the patients was 12 years (range: 4-15.8). Five had atrial tachycardia, two had ventricular tachycardia, the others had Mahaim fiber tachycardia, permanent junctional reciprocating tachycardia and atrioventricular reentrant tachycardia. Seven patients had catheter ablation and three patients who had previous heart surgery were treated with antiarrhythmic drugs. Median ejection fraction was 33% (range: 10-48), median left ventricle end-diastolic diameter was 55 mm (range: 30-78). All showed complete recovery with median ejection fraction 60% (range: 55- 78). Two patient with severe heart failure required extracorporeal membrane oxygenation support, one of them had ventricular assist device support but the device was removed after successful ablation. After two years this patient required permanent pacemaker implantation due to complete atrioventricular block. Tachycardia-induced cardiomyopathy is a rare and treatable cause of heart failure. Early recognition is critical, aggressive treatment aimed at controlling the arrhythmia results in symptom resolution and recovery of ventricular function.