The effect of albumin and total protein values as nutritional markers after Fontan surgery on prognosis.

INTRODUCTION: Children with CHD develop heart failure due to increased pulmonary blood flow, cyanosis, and pulmonary hypertension. The metabolic needs of these children differ from those of healthy children, and malnutrition is common. Protein-losing enteropathy has been reported in 5 to 13% of patients after the Fontan procedure. Serum albumin and total protein levels, which are indicators of the quality of post-operative care, can be useful tools for monitoring and examining the intensive care treatment strategies of these patients. In our retrospective study, the effects of albumin and total protein values, which are two of the markers that give us an idea about diet, nutritional status, and inflammation, on the prognosis of children who underwent the Fontan procedure were investigated. METHOD: In our study, 127 patients who underwent Fontan procedure in our clinic between 2012 and 2021 were analysed retrospectively. Of the patients, 52.7% (n = 67) were male and 47.3% (n = 60) were female. The mean age is 5.83 ± 4.63 years. Patients who underwent albumin replacement were not included in the study. RESULTS: Although the relationship between pre-operative albumin and total protein values and post-operative mortality was not statistically significant, the inverse correlation of post-operative albumin 1st, 2nd, and 3rd-day values and post-operative total protein 1st, 2nd, and 3rd-day values with mortality was found to be statistically significant. In addition, we found that mortality was statistically high in patients whose total protein amount was below 6.65 mg/dl in the early post-operative period. CONCLUSION: Albumin and total protein, whose blood levels can vary with diet, can be used as predictors in the early post-operative prognosis of Fontan patients. In addition, when we examined the exitus patients, it was observed that the total protein amount was below 6.65 mg/dl on the post-operative 1st day. Based on this, we think that a diet with high protein content before surgery will help reduce post-operative early mortality.

The prognostic significance of early troponin levels in patients undergoing aortic ridge surgery.

Subaortic stenosis is a CHD that can lead to left ventricular hypertrophy, heart failure, and aortic valve damage if left untreated. The gold standard treatment for subaortic stenosis is septal myectomy. However, there is no clear consensus on the surgical margins required for adequate muscle resection. In this retrospective study, we reviewed the records of 83 patients who underwent subaortic stenosis surgery between 2012 and 2020 to investigate the effect of early troponin levels on prognosis. We excluded patients with additional cardiac pathologies, hypertrophic obstructive cardiomyopathy, and valvular aortic stenosis.Troponin levels were recorded in the early post-operative period, and patients were monitored for complications such as ventricular arrhythmia, left ventricular systolic dysfunction, infective endocarditis, and pacemaker implantation. The troponin levels were significantly higher in the patients who had septal myectomy. The degree of myectomy affected the risk of complications in the early post-operative period and recurrence in the later period. However, when the gradient was substantially or completely removed by myectomy, patients experienced significant symptom improvement in the early post-operative period, and their late survival was equivalent to that of healthy individuals of the same age.Our findings suggest that monitoring troponin levels in patients undergoing septal myectomy may be beneficial in predicting the risk of complications. However, further studies are needed to establish the optimal surgical technique and extent of muscle resection required for subaortic stenosis treatment. Our study adds to the existing knowledge of the benefits and risks associated with septal myectomy as a treatment option for subaortic stenosis.

Left ventricular dysfunction in a child with asymptomatic pre-excitation: Cure with catheter ablation.

Wolff-Parkinson-White syndrome, is known to cause left ventricular dysfunction or dilated cardiomyopathy secondary to sustained tachycardia in infants and children. However, left ventricular dysfunction secondary to pre-excitation related abnormal ventricular activation has been reported in a limited number of cases. This condition should be recognized early, as catheter ablation of the accessory pathway can permit rapid ventricular function improvement. In this paper, we present a 2.5-year-old patient diagnosed with tachycardia-free Wolff-Parkinson-White syndrome with a right free wall accessory pathway and depressed cardiac function, whose left ventricular function is completely restored after successful catheter cryoablation.

Evaluation of the relationship between cardiopulmonary exercise test findings and clinical status in children and adolescents with congenital heart disease.

BACKGROUND: The cardiopulmonary exercise test is accepted as a helpful diagnostic tool in risk stratification, evaluation of prognosis, and guiding treatment modality in adults with congenital heart disease. In this study, we present our experience with the use of cardiopulmonary exercise test in children with congenital heart disease in different physiological and anatomical classifications. METHODS: In this retrospective study, 25 children and adolescents who applied to the pediatric cardiology outpatient clinic between 2017 and 2020 with the diagnosis of different types of congenital heart disease were included. Demographic characteristics, electrocardiogram, echocardiogram, cardiopulmonary exercise test, spirometry, pro-BNP values, and in selected 20 patients; cardiac MRI data were examined. The modified Ross classification was used for heart failure grading. RESULTS: The mean age of the patients was 14.8 ±2.39 years. Fifteen (60%) of the patients were male and 10 (40%) were female. In the modified Ross classification, patients in group I-II had significantly higher maximum exercise time, heart rate reserve %, peak VO2, and VO2/kg values compared to those in group III (p=0.026, p=0.007, p=0.043, p= 0.018, respectively). Cardiopulmonary exercise test and spirometry values obtained from the patients were evaluated in the light of clinical and other laboratory findings, and surgical/interventional treatment was decided for 4 patients with the use of these test results. CONCLUSIONS: Cardiopulmonary exercise test is a useful noninvasive diagnostic tool in guiding the treatment decision and predicting the prognosis of pediatric patients with congenital heart disease, who have borderline symptoms.

Vascular Wall Changes and Arterial Functions in Children with Surgically Repaired Aortic Coarctation.

OBJECTIVE: We investigated arterial functions by measuring carotid-intima-media thickness, flow-mediated dilatation of the brachial artery, and distensibility and stiffness of the abdominal aorta as early indicators of cardiovascular risk in children followed up after coarctation repair. MATERIALS AND METHODS: Twenty patients with successful repair of coarctation and 27 healthy children were investigated. Two-dimensional echocardiographic images, and carotid and brachial ultrasound studies were performed. RESULTS: The ages of the study group ranged from 5.3 to 22 years, and those of the control group from 7 to 17 years. The age at time of surgery was between 0.23 and 257 months. Average follow-up duration after repair was between 11 and 257 months. The stiffness index of the abdominal aorta was significantly higher in patients with coarctation repair than controls (mean = 0.625 ± 0.41, mean = 0.11 ± 0.73; P = .007). Flow-mediated dilatation of the brachial artery in the first minute decreased significantly among the patients (mean = 4.5 ± 2.7, mean = 6.9 ± 4.5; P = .005). Age of the patients had a negative correlation with distensibility of the abdominal aorta (r = -0.572; P = .008) and a positive correlation with stiffness of abdominal aorta (r = 0.566, P = .009). CONCLUSION: This study suggested that vascular wall changes in children and adolescents can be seen even after successful coarctation repair and may progress toward overt atherosclerosis at older ages.

Platypnea-orthodeoxia syndrome in a child: relief of symptoms after transcatheter closure of patent foramen ovale.

Platypnea-orthodeoxia syndrome is a rare clinical entity characterised by positional dyspnoea and arterial desaturation while in the upright position, the symptoms generally occurring in adults. We describe a 12-year-old girl diagnosed with platypnea-orthodeoxia syndrome associated with patent foramen ovale. The symptoms resolved following percutaneous patent foramen ovale closure.

Evaluation of Arterial Functions and Carotid Intima Media Thickness in Children During Mid-Term Follow-Up After Kawasaki Disease.

OBJECTIVE: Kawasaki disease (KD) is a multisystemic vasculitis of medium- and small-sized arteries. The involvement of the coronary arteries may lead to long-term cardiovascular sequelae. We studied the elasticity of the aorta and the common carotid artery (CCA), flowmediated dilatation of the brachial artery, and carotid intima media thickness, as well biomarkers such as high-sensitivity C-reactive protein (hs-CRP) and elastin, as useful indicators of cardiovascular risk in patients, following KD. METHODS: The study group consisted of 26 patients with a history of KD, and 26 healthy children. Echocardiography, and carotid and brachial ultrasound studies were performed. Plasma hs- CRP and elastin levels were studied in both groups. RESULTS: The stiffness indices (SI) obtained from the aortic arch, abdominal aorta, and the CCA were increased in the patients, compared to the controls. Distensibility was decreased at the sinus of Valsalva, the sinotubular junction, the aortic arch, and the ascending aorta, compared to the control group. Flow-mediated dilatation (FMD) was lower in the patients than in the controls. The age of the patients had a negative correlation to distensibility of the aortic arch and abdominal aorta, and a positive correlation to the stiffness of the aortic arch. Follow-up duration correlated positively to stiffness of the aortic arch. Carotid intima-media thickness (CIMT), serum hs-CRP, and elastin levels did not differ between the groups. CONCLUSION: Increased aortic and carotid stiffness and decreased aortic distensibility suggest impaired arterial functions following KD. Long-term follow-up and monitorization early in cardiovascular disease is needed.

Pacemaker lead-induced tricuspid stenosis treated with percutaneous valvotomy.

BACKGROUND: Tricuspid stenosis is an uncommon complication of ventricular pacemaker electrode implantation, with few cases reported in the literature. CASE: We present an 18-year-old male who developed severe tricuspid stenosis 15 years after endocardial VVI pacemaker implantation for complete AV block following a surgically repaired ventricular septal defect. CONCLUSION: In this case we have shown that percutaneous balloon valvuloplasty was performed and successful in treating this complication.

Transcatheter baffle leak closure via an amplatzer septal occluder in a senning-operated child.

Senning operation is a surgical treatment for transposition of great arteries that can be complicated by post-procedural atrial or caval baffle leaks. We present a 6-year-old boy with a history of Senning repair for transposition of great arteries, who developed a pulmonary venous baffle leak. Percutaneous baffle leak closure was successfully performed using an Amplatzer septal occluder.

Aortic elasticity and the influence of valve morphology in children with bicuspid aortic valve.

AimWe investigated dimensions and elasticity of whole aorta in patients with bicuspid aortic valve and influence of valve phenotype. METHOD: The study group included 44 patients and 42 controls. Patients were divided into groups according to the type of valve opening as horizontal - fusion between right and left coronary cusps - and vertical - fusion between right-non-coronary cusps; according to age they were divided into younger (5-10 years) and older patients (11-16 years). Our study did not include valve phenotype with fusion between left and non-coronary cusps. Systolic-diastolic diameters of aortic annulus, sinus valsalva, sinutubular junction, arcus, and ascending-descending and abdominal aorta were measured and z-scores were obtained. Aortic strain, distensibility, and stiffness index were calculated. Flow-mediated dilatation of brachial artery was studied. RESULTS: z-Scores at annulus, sinus valsalva, sinutubular junction, and ascending aorta were higher in study patients (p=0.001, p=0.0001, p=0.0001, p=0.0001, respectively). z-Scores of sinus valsalva and sinotubular junction were higher in the horizontal group than in the vertical group (p=0.006, p=0.023, respectively). z-Score was over +2 in 51% of patients with horizontal morphology and 33% of patients with vertical morphology (p=0.0001). Ascending aorta was more distensible and less stiff in the study group (11.3±5.63 versus 7.91±4.5, p=0.002; 4.76±3.60 versus 6.19±3.44 cm2.dyn-1.10-6, p=0.033, respectively). Stiffness index of ascending, arcus, and abdominal aorta were higher in the horizontal group (p=0.004, p=0.038, p=0.006, respectively). Ascending aorta was more distensible and less stiff in the younger group (p=0.007, p=0.027, respectively) but did not differ in the older group compared with the control group. CONCLUSION: Aortic dimensions are enlarged in patients with bicuspid aortic valve starting from childhood, suggesting the presence of generalised aortopathy. Aortic elasticity is increased at young age and decreased with age.

Aortic elasticity and carotid intima-media thickness in children with mitral valve prolapse.

Aim We aimed to study the dimensions, systolic and diastolic functions of the left ventricle; dimensions and elasticity of the aorta; and carotid intima-media thickness and flow-mediated dilatation of the brachial artery in mitral valve prolapse. METHODS: The study group consisted of 43 patients (mean age=13.3±3.9) and 42 healthy children (mean age=12.9±3.4). Left ventricular end-diastolic, end-systolic, left atrial diameters, interventricular septum, and left ventricular posterior wall thickness were measured. Ejection and shortening fractions were calculated by M-mode. Measurements were adjusted to the body surface area. Mitral annulus, and systolic and diastolic diameters of the aortic annulus and aorta at each level were obtained; z-scores, aortic strain, distensibility, stiffness index were calculated. Carotid intima-media thickness and flow-mediated dilatation were studied. Patients were classified as classical/non-classical mitral valve prolapse and younger/older patients. RESULTS: Left ventricular end-systolic, end-diastolic, and left atrial diameters (p=0.009, p=0.024, p=0.001) and aortic z-scores at annulus, sinus valsalva, and sinotubuler junction were larger (p=0.008, p=0.003, p=0.002, respectively) in the mitral valve prolapse group. Aortic strain and distensibility increased and stiffness decreased at the ascending aorta in the patient group (p=0.012, 0.020, p=0.019, respectively). Classical mitral valve prolapse had lower strain and distensibility and higher stiffness of the aorta at sinus valsalva level (p=0.010, 0.027, 0.004, respectively). Carotid intima-media thickness was thinner in the patient group, especially in the non-classical mitral valve prolapse group (p=0.037). Flow-mediated dilatation did not differ among the groups. CONCLUSION: Mitral valve prolapse is a systemic disease of the connective tissue causing enlarged cardiac chambers and increased elasticity of the aorta. Decreased carotid intima-media thickness in this group may indicate low atherosclerosis risk.

Ductal closure with intravenous paracetamol: a new approach to patent ductus arteriosus treatment.

OBJECTIVES: Indomethacin and ibuprofen are commonly used in the treatment of hemodynamically significant patent ductus arteriosus (hsPDA). These drugs are associated with serious adverse events, including gastrointestinal perforation, renal failure and bleeding. The role of paracetamol has been proposed for the treatment of PDA. METHODS: We report a series of 11 neonates (birth weight: 415-1580 g; gestational age: 23-30.3 weeks) who were treated with paracetamol for a hsPDA. Neonates with hsPDA were treated with paracetamol in the presence of contraindications to ibuprofen or indomethacin. The condition of significant PDA was defined by the presence of at least one of the following criteria: internal ductal diameter # 1.4 mm/kg body weight, left atrium (LA)-to-aortic (Ao) root ratio > 1.4, unrestrictive pulsatile transductal flow, reverse or absent diastolic flow in the descending aorta along with clinical findings. Intravenous (IV) paracetamol was given at doses 15 mg/kg every 6 h for three days. RESULTS: Successful ductal closure was achieved in 10 out of 11 babies (90.9%). No adverse or side effects were observed during the treatment. CONCLUSIONS: On the basis of these results, paracetamol could be considered as a promising and safe therapy for the treatment of PDA in preterm infants.

Embolization of a complex pulmonary arteriovenous fistula and coarctation treatment with covered stent at the same session.

Pulmonary arteriovenous fistula (PAVF) are rare malformations that may cause serious complications such as paradoxical embolism, stroke, pulmonary hemorrhage and hemoptysis. Accompanying cardiac malformations such as aortic coarctation were not reported previously. Here we present a case of complex PAVF associated with aortic coarctation. The patient was treated successfully by transcatheter embolization of PAVF with amplatzer vascular plug I and II and implantation of a covered CP stent for coarctation at the same session.

Coronary arteriovenous fistula causing hydrops fetalis.

Fetal heart failure and hydrops fetalis may occur due to systemic arteriovenous fistula because of increased cardiac output. Arteriovenous fistula of the central nervous system, liver, bone or vascular tumors such as sacrococcygeal teratoma were previously reported to be causes of intrauterine heart failure. However, coronary arteriovenous fistula was not reported as a cause of fetal heart failure previously. It is a rare pathology comprising 0.2-0.4% of all congenital heart diseases even during postnatal life. Some may remain asymptomatic for many years and diagnosed by auscultation of a continuous murmur during a routine examination, while a larger fistulous coronary artery opening to a low pressure cardiac chamber may cause ischemia of the affected myocardial region due to steal phenomenon and may present with cardiomyopathy or congestive heart failure during childhood. We herein report a neonate with coronary arteriovenous fistula between the left main coronary artery and the right ventricular apex, who presented with hydrops fetalis during the third trimester of pregnancy.