Staged treatment of a premature newborn with interrupted aortic arch and aorta-pulmonary window using intraoperative hybrid procedure before subsequent total correction - Long-term follow-up.

Interrupted aortic arch (IAA) associated with an aortopulmonary window (APW) is a rare cardiac malformation that needs an immediate diagnosis after birth and surgical treatment to avoid irreversible pulmonary lesions. Herein, we describe a case of successful staged treatment of premature neonate using many operative procedures. The first stage was stenting of patent ductus arteriosus with banding of pulmonary artery branches using a hybrid approach. At the age of one, he underwent total correction of malformation using Gore-Tex patch. Two procedures of implantation and redilatation of the previously implanted stents were performed in the following years. In the 8-year follow-up, the patient has been in good clinical condition, without cardiac symptoms. To the best of our knowledge, this is the first case report representing such a long-term follow-up of treatment of IAA accompanied by APW.

Coronary steal phenomenon following right ventricle decompression and revascularization of atretic left coronary ostium: case report.

BACKGROUND: Coronary steal phenomenon and myocardial ischemia is a complication following decompression of a hypertensive right ventricle in patients with left coronary-cameral fistulae. CASE PRESENTATION: We present a 12-year-old girl with a complex heart defect successfully operated on using a hybrid surgical-interventional approach to decompress the ventricle, embolize the fistula and reconstruct the atretic left coronary ostium. CONCLUSIONS: A novel hybrid strategy is the best solution for coronary-cameral fistulas reliant on high ventricular pressure at high risk for coronary steal phenomenon.

Hypoplastic left heart syndrome: from the prenatal to the postnatal period.

OBJECTIVES: To analyse a population of foetuses with prenatally diagnosed hypoplastic left heart syndrome (HLHS). MATERIAL AND METHODS: Retrospective study of foetuses diagnosed with HLHS between 2013 and 2017 in a referral centre. RESULTS: HLHS was found in 9.7% (65/665) of foetuses with cardiovascular abnormalities (CVA). As an isolated anomaly, HLHS was present in 40% of cases; in 24.5% other CVA were detected; in 14%, CVA and extracardiac anomalies; and in 21.5% only extracardiac malformations. Genetic disorders were present in 18.4% (12/65) of foetuses. 42% of cardiovascular and 25% of extracardiac anomalies were diagnosed postnatally. There were 10 (15.4%) elective terminations, 1 (1.5%) spontaneous foetal demise. Two newborns died after birth before surgery. Of the 52 children who underwent Norwood surgery, 13 (25%) died (9 with additional anomalies, and 4 with isolated HLHS). Of the 38 children who underwent stage II surgery, 2 (5.2%) with isolated HLHS died, and 1 (2.6%) with CVA. CONCLUSIONS: A diagnosis of HLHS is an indication for a detailed examination of cardiac and noncardiac structures. It is advisable to consider genetic testing, together with the microarray assessment. The prognosis depends on underlying cardiac and extracardiac anomalies and coexisting genetic defects.

Recent achievements in transcatheter closure of ventricular septal defects: a systematic review of literature and a meta-analysis.

BACKGROUND: Ventricular septal defect (VSD) is one of the most common congenital heart defects. Currently, surgery remains the treatment of choice. However, transcatheter techniques for closing of various types of VSDs have become an alternative. AIMS: The objective of our study was to present the outcomes of transcatheter closure of various types of VSD based on a systematic review of recent publications. METHODS: A systematic review of studies published in English between January 2014 and March 2020 was performed using the PubMed database (MEDLINE) independently by 2 reviewers. Data on success and complication rates were extracted. Studies including fewer than 5 patients and those with acquired VSD were excluded from the analysis. RESULTS: Finally, 44 studies were included for analysis, with a total number of 4050 patients. The pooled estimate of the overall success rate based on the random effects model was 97.96% (95% CI, 97.37-98.56; Q test P 0.99; I 2 = 0%) for permanent VSD. CONCLUSIONS: Transcatheter closure of selected VSDs appears to be an effective and safe method of treatment. Recent studies have shown high rates of successful interventions with a low incidence of complications.

Pericarditis with cardiac tamponade - A misleading complication of appendicitis: A rare case report.

INTRODUCTION: As severe complication of appendicitis as effusive pericarditis is a rare phenomenon. Taking into consideration open access data in a 50-year period we perceived three pediatric cases with pericarditis as a complication of appendicitis. PRESENTATION OF CASE: We report a 15-year-old boy who presented cardiac tamponade, bilateral pleural effusions, liver enlargement and ascites as a presentation of atypical appendicitis. DISCUSSION: Even nowadays appendicitis could be difficult to diagnose in children. A delayed diagnosis could be responsible for severe morbidity and mortality. CONCLUSION: This report is aimed to be a reminder of serious life-threatening complications after a common abdomen condition - appendicitis.

Echocardiography and Newer Imaging Techniques in Diagnosis and Long-Term Follow-Up of Primary Heart Tumors in Children.

BACKGROUND: Primary heart tumors (PHTs) in the pediatric population are very rare and do not manifest any characteristic symptoms. METHODS: A retrospective analysis of 61 cases was undertaken. Data from three centers for the years 2003-2018 were gathered. The tumors' clinical course, location, number, hemodynamic, treatment, and follow-up were evaluated. Echocardiography was complemented with magnetic resonance imaging, computer tomography, and histopathological examination. RESULTS: Out of 61 PHT diagnoses, 56 (91.8%) were circumstantial including all 16 (26.2%) prenatal tumors. The reasons for cardiological consultations were arrhythmia, syncopes, lowered physical performance, and murmurs. Only five patients (8.2%) were suspected of tumors based on previous symptoms of sclerosis tuberosa. Rhabdomyoma was the most frequently found PHT (60.7%). The tumors were predominantly located in the ventricles (49.1%) and intraventricular septum (14.9%) and tended to be single (70.5%). About 37.7% of patients suffered from coexistent multi-organ problems, two (3.28%) from congenital heart defects and one (1.64%) from Carney's syndrome. Tumor resection was performed on 26 (42.7%) patients, of which 16 (61.5%) underwent total and 10 (38.5%) partial tumor resection. During the follow-up (mean 4.3 years), 54 patients (88.5%) have improved or were stable, while seven (11.5%) died. CONCLUSIONS: Primary pediatric heart tumors are diagnosed completely circumstantially, and the most common is rhabdomyoma, although arrhythmia may suggest fibroma. Diagnosis of a heart tumor in children is not synonymous with fatal prognosis, and most of them require only constant observation. Life-saving operation allows improvement, while the prognosis for malignant tumors in children is definitely unfavorable.

Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL).

We present the results from the pediatric arm of the Polish Registry of Pulmonary Hypertension. We prospectively enrolled all pulmonary arterial hypertension (PAH) patients, between the ages of 3 months and 18 years, who had been under the care of each PAH center in Poland between 1 March 2018 and 30 September 2018. The mean prevalence of PAH was 11.6 per million, and the estimated incidence rate was 2.4 per million/year, but it was geographically heterogeneous. Among 80 enrolled children (females, n = 40; 50%), 54 (67.5%) had PAH associated with congenital heart disease (CHD-PAH), 25 (31.25%) had idiopathic PAH (IPAH), and 1 (1.25%) had portopulmonary PAH. At the time of enrolment, 31% of the patients had significant impairment of physical capacity (WHO-FC III). The most frequent comorbidities included shortage of growth (n = 20; 25%), mental retardation (n = 32; 40%), hypothyroidism (n = 19; 23.8%) and Down syndrome (n = 24; 30%). The majority of children were treated with PAH-specific medications, but only half of them with double combination therapy, which improved after changing the reimbursement policy. The underrepresentation of PAH classes other than IPAH and CHD-PAH, and the geographically heterogeneous distribution of PAH prevalence, indicate the need for building awareness of PAH among pediatricians, while a frequent coexistence of PAH with other comorbidities calls for a multidisciplinary approach to the management of PAH children.

Malignant ventricular arrhythmias and other complications of untreated accessory pathways: an analysis of prevalence and risk factors in over 600 ablation cases.

BACKGROUND: The presence of accessory pathways (APs) is a risk factor for sudden cardiac death and other clinical complications. AIMS: We aimed to characterize all adverse events likely related to the presence of APs in patients referred for AP ablation and to identify risk factors for malignant arrhythmias. METHODS: We performed a retrospective analysis of consecutive patients referred for AP ablation from 2002 to 2017. Electrocardiograms, electrophysiological system records, and hospital discharge notes were reviewed. We collected data concerning symptoms before ablation, occurrence of ventricular fibrillation or malignant atrial fibrillation (AF), as well as other complications related to APs. RESULTS: We identified 602 patients with APs. Serious AP­related events were observed in 41 patients, including 14 sudden cardiac arrests (1 death) and 16 pre­cardiac arrest events. Other complications included strokes, pulmonary edema, heart failure, and unnecessary device implantation. The risk of malignant arrhythmias decreased with a longer shortest preexcited RR interval (per 10 ms: odds ratio [OR], 1.3; 95% CI, 1.16­1.47) and increased with age (per 10 years: OR, 1.29; 95% CI, 1.06­1.57). The presence of inducible AF, but not sole atrioventricular reentrant tachycardia, increased the risk for malignant arrhythmias when compared with patients without any inducible arrhythmias. CONCLUSIONS: Patients with APs referred for ablation commonly present with various adverse events. The predictive value of clinical risk factors for malignant arrhythmias is too low to prevent devastating consequences. When high safety and efficacy of AP ablation are ensured, even a low risk of sudden death is unacceptable and a lower threshold for prophylactic ablation should be used to prevent AP­related adverse events.

Clinical Evaluation of Exercise Capacity in Adults with Systemic Right Ventricle.

The right ventricle provides systemic circulation in individuals with congenitally corrected transposition of the great arteries (CCTGA) and in those with complete transposition who have had an atrial switch repair (DTGA). The aim of this study was to evaluate how the systemic right ventricle adapts to increased workload and oxygen demand during exercise. From November 2005 through December 2015, 3,358 adult patients with congenital heart disease were treated at our institution; we identified 48 (26 females, 22 males; median age, 25.4 ± 8.1 yr) who met the study criteria; 37 had DTGA and atrial switch repair, and 11 had CCTGA. We studied their echocardiographic and cardiopulmonary exercise test results. A control group consisted of 29 healthy sex- and age-matched volunteers. On exercise testing, oxygen uptake at anaerobic threshold, peak oxygen uptake, peak heart rate, and percentage of maximal heart rate were significantly lower in the group with systemic right ventricle than in the control group (all P <0.001); in contrast, the peak ventilatory equivalent for carbon dioxide was higher in the study group (P=0.013). Impaired systemic right ventricular function reduced peak oxygen uptake. The peak heart rate was lower in the CCTGA group than in the DTGA group. Our results indicate that reduced exercise capacity is related to impaired systemic right ventricular function, severe tricuspid valve regurgitation, and chronotropic incompetence. There was no correlation between cardiopulmonary exercise test results and time after surgery. Chronotropic efficiency is lower in individuals with CCTGA than in those with DTGA.

Noninvasive assessment of liver status in adult patients after the Fontan procedure.

INTRODUCTION A hemodynamic derangement in Fontan circulation causes liver pathology known as Fontan­­associated liver disease. Although liver biopsy is a standard for diagnosis of liver fibrosis, noninvasive methods are being developed, including shear wave elastography (SWE). OBJECTIVES We aimed to evaluate the degree of liver stiffness (LS) using SWE in patients with Fontan circulation in a long­­term follow­­up and to investigate a relationship between patient characteristics and LS. PATIENTS AND METHODS The study included 59 patients after the Fontan procedure. Clinical examinations and laboratory tests were performed. According to the stage of LS, patients were divided into 2 subgroups: group 1 (METAVIR stages F1 and F2) and group 2 (METAVIR stages F3 and F4). The ratio of aspartate transaminase to alanine transaminase, aspartate transaminase­­to­­platelet ratio index (APRI), fibrosis­­4 (FIB­­4) score, and Forns index were assessed. RESULTS The median LS was 9.1 kPa (interquartile range, 3.9-18.5 kPa). Five patients (9%) demonstrated LS in stage F1; 14 (26%), F2; 28 (52%), F3; and 7 (13%), F4. Group 2 had significantly higher aspartate transaminase and γ­­glutamyltranspeptidase levels, APRI, FIB­­4, and Forns index, and lower platelet count than group 1. A canonical correlation analysis indicated that LS and thrombocytopenia were related to time from the Fontan procedure, age at procedure, and single ventricular ejection fraction. CONCLUSIONS We showed that adult patients after the Fontan procedure develop liver dysfunction. Time from surgery, age at procedure, and single ventricular ejection fraction are related to the degree of LS assessed by SWE. Finally, SWE, APRI, Forns index, and FIB­4 score may help assess the degree of liver fibrosis.

Long-term observation of adults after successful repair of aortic coarctation.

INTRODUCTION: Despite successful repair of aortic coarctation, cardiovascular complications occur. AIM: To analyse type and frequency of late complications and their impact on exercise capacity in adults after aortic coarctation repair. MATERIAL AND METHODS: Fifty-eight adults after aortic coarctation repair, 36 male, median age 27.46 ±10.57, were compared to 30 healthy volunteers. Physical examination, transthoracic echocardiography, carotid intima-media thickness measurement, cardiopulmonary exercise test and 24-hour ambulatory blood pressure monitoring were performed. RESULTS: The main complications were: arterial hypertension 48.3%, myocardial hypertrophy in echocardiography 29.34%, recoarctation 25.86%, aortic dilation 13.79% and coronary artery disease 6.89%. Exercise tolerance was reduced in the cardiopulmonary exercise test. The VO2/kg peak was lower, 29.01 ±8.79 vs. 49.16 ±7.38 ml/kg/min, p < 0.001, VE/VCO2 peak higher 28.18 ±4.69 vs. 26.78 ±3.13, p = 0.017. The peak heart rate was reduced, 157.28 ±22.22 vs. 177.93 ±23.08 bpm, p < 0.001, peak systolic blood pressure was higher, 174.79 ±17.62 vs. 153.33 ±4.79 mm Hg, p < 0.001. Systolic blood pressure in 24-hour ambulatory monitoring correlated with left ventricle mass index, r = 0.29, p = 0.025, wall thickness, r = 0.31, p = 0.039. Age at operation was related to left ventricle wall thickness, r = 0.27, p = 0.041, and carotid intima-media thickness, r = 0.26, p = 0.046. There was no association of any cardio-pulmonary parameters with time from surgery, type of operation or echocardiography results. CONCLUSIONS: Adults after aortic coarctation repair suffer from arterial hypertension, recurrent aortic stenosis, aortic aneurysms, and coronary artery disease. Reduced exercise capacity in cardio-pulmonary exercise test is related to hypertensive reaction and chronotropic incompetence.

Quality of life in adults with repaired tetralogy of Fallot.

INTRODUCTION: Observations of patients after repair of tetralogy of Fallot (ToF) indicate good correction results and long-term survival. Few papers have been published in which the quality of life (QoL) of this population has been assessed. AIM: To evaluate QoL in adults with repaired ToF.. MATERIAL AND METHODS: We included 39 patients with repaired ToF and 40 age- and sex-matched healthy volunteers. Information recorded included echocardiography, cardiac magnetic resonance, cardiopulmonary exercise test, and self-reported health-related QoL questionnaire (SF-36).. RESULTS: The perceived physical and mental domains of health were signi cantly poorer in ToF patients than in controls. A positive correlation between VO2 peak and physical domains was observed: (VO2 peak vs. physical domains (r = 0.6, p ≤ 0.001), general health (r = 0.36, p = 0.03), and physical complex status (r = 0.51, p = 0.001). VO2 peak % correlated with physical functioning (r = 0.43, p = 0.007), general health (r = 0.39, p = 0.015) and physical complex status (r = 0.49, p = 0.002). Right ventricle ejection fraction, determined with cardiac magnetic resonance, positively correlated with role physical (r = 0.38, p = 0.04). In echocardiography, pressure half time was posi- tively correlated with physical functioning (r = 0.48, p = 0.004) and role physical (r = 0.4, p = 0.02).. CONCLUSIONS: The QoL in adults after repair of ToF and healthy control subjects was compared directly. The self-perceived physical and mental domains of health were significantly poorer in ToF patients than in controls. Strong associations were found between objective exercise capacity and physical aspects of quality of life. Complex assessment and quality of life instruments should be used together to obtain an accurate view of health status of patients with repaired ToF.

Long-Term Effects of Percutaneous Fenestration Following the Fontan Procedure in Adult Patients with Congenital Univentricular Heart.

BACKGROUND The Fontan procedure, performed for univentricular heart, may also include the technique of percutaneous fenestration to create a small atrial septal defect (ASD) and a right-to-left shunt. The aim of this study was to evaluate the long-term effects of fenestration in adult patients who had a Fontan procedure for univentricular heart. MATERIAL AND METHODS Fontan surgery was performed in 39 patients, including 19 (49%) patients with fenestration (Group I), and 20 (51%) patients without the fenestration procedure (Group II). Laboratory tests in both groups included echocardiography, plethysmography, cardiopulmonary exercise testing, and 24-hour Holter monitoring. RESULTS Compared with patients in Group I, patients in Group II had a significantly increased level of N-terminal pro-brain natriuretic peptide (NT-proBNP) (p=0.04), alkaline phosphatase (ALP) (p=0.01) and a significant increase in frequency of atrial fibrillation (p=0.04). Patients in Group I had a significantly increased systemic ventricular ejection fraction (SVEF) (p=0.05) and increased heart rate (HR) (p=0.006), heart rate reserve (HRR) (p=0.02), ventilatory equivalent (VE) (p=0.01), and VO2 peak (p=0.05) on cardiopulmonary exercise testing (CPET). Renal, hematologic, and ventilatory parameters, and incidence of thromboembolism showed no significant differences between the groups. CONCLUSIONS Long-term follow-up of patients who underwent Fontan procedures with percutaneous fenestration had improved single ventricular function, lower NT-proBNP levels, improved exercise capacity, and reduced ALP levels. These findings indicate that percutaneous fenestration closure should be considered for adult patients who have undergone Fontan procedure for univentricular heart.

What determines the quality of life of adult patients after Fontan procedure?

BACKGROUND: Despite the low early mortality of Fontan procedures, Fontan patients are prone to vari-ous cardiac and extra-cardiac complications in the long term. This may influence patient perception of their health and outcome. The aim of the study was to assess the relationship of multi-organ compli-cations and physical efficiency with self-reported health-related quality of life (QOL) in adult Fontan patients. METHODS: Quality of life was assessed with the Short Form-36 questionnaire. Laboratory tests were done together with echocardiography, plethysmography, and cardiopulmonary exercise test. RESULTS: The QOL was poorer in patients than in control subjects. The physical characteristics of patients correlated with dynamic ventilatory parameters, heart rate at the peak of exercise, alanine aminotransferase and albumin level. CONCLUSIONS: Liver impairment and chronotropic incompetence during exercise are associated with poor QOL in patients after Fontan procedure. In these patient, hepatic, pulmonary and cardiac functions should be carefully monitored. (Cardiol J 2018; 25, 1: 72-80).