Inguna Lubaua

Follow‐up of the patients after stem cell transplantation for pediatric dilated cardiomyopathy

Dilated cardiomyopathy is a serious problem in pediatric cardiology. Despite the relatively low incidence, the mortality is high. The conservative therapy does not improve the prognosis, and possibilities of heart transplantation are limited. There are multiple trials of use of stem cells for ischemic heart disease in the adult population. This allows us to believe that the method has perspectives in pediatric cardiology. We performed the cell therapy for seven patients, six of them had complete one yr follow‐up after procedure. Five to 30 milliliters of bone marrow was aspirated from the iliac crest and 17 to 122 million BMCs were isolated. The average basal EF was 33.5%. We observed increasing of EF up to 54% (=9.54, p=0.00154) in a 6‐month period and up to 54.5% (=10.82, p= 0.00315) after one yr. The changes of LVEDV also were observed. The LVEDV decreased in average per 13.05%. There were no observed side effects or heart rhythm disorders. Intramyocardial administration of bone marrow‐derived progenitor cells proved to be a technically feasible and safe method. Up until now, the results obtained have been promising and we suppose that bone marrow‐derived progenitor cell intramyocardial transplantation can be used.

Giant fibroma of the right ventricle

Cardiac fibromas are rare benign tumours of connective tissue that occur most frequently in children within the left ventricle. Spontaneous regression has not been observed, and surgical intervention is usually required. We have successfully treated a 1-year old girl with a giant fibroma of the right ventricle using the principles of the Batista procedure. The diagnosis was primarily made using transthoracic echocardiography.

Incidence and Outcomes of Patients with Functionally Univentricular Heart Born in Latvia, 2007 to 2015

Background and Objectives: A functionally univentricular heart is the term used to describe congenital heart defects where it is impossible to restore two pumping chambers. These lesions are associated with high mortality, morbidity, and medical resource utilization. The aim of this study was to review incidence and outcomes of patients with a functionally univentricular heart at the only pediatric cardiac surgery center in Latvia. Methods: We performed a retrospective review of medical records of (i) all children with a functionally univentricular heart treated at the Clinic of Pediatric Cardiology and Cardiac Surgery, and (ii) all prenatally diagnosed cases of univentricular heart at Children’s Clinical University Hospital in Latvia. We reviewed data regarding children born from January 1, 2007, to December 31, 2015. The children’s cardiac anatomy and interventions were categorized in accordance with the International Pediatric and Congenital Cardiac Code (v3.3). Results: During the study period, 49 patients with a functionally univentricular heart were admitted to Children’s Clinical University Hospital with a corrected incidence of 0.69 per 1000 live births per year. There were 26 patients that had a hypoplastic left ventricle, and 22 patients that had a hypoplastic right ventricle, while one patient had an indeterminate ventricle. Thirty (61.2%) patients had died by the end of data collection. Twenty-one of the 30 deaths occurred before or immediately after stage I surgical palliation. Cumulative neonatal and 5-year survival of patients with a hypoplastic right ventricle was 81.8% and 63.6%, respectively; for patients with hypoplastic left ventricle—46.2% and 17.3%, respectively. Discussion: This is the first mid-term outcome study of patients with a univentricular heart in Latvia. The high mortality reflects the challenges of a small-volume, developing congenital cardiac surgery center. Data from this study will be used as a baseline for quality improvement.

Echocardiographic Follow up After Surgical Correction of Aortic Coarctation During The First Year of Life

Abstract Coarctation of the aorta (AoCo) accounts for 6–10% of congenital heart diseases in infants. We analysed echocardiographic findings of patients operated on for AoCo in the University Hospital for Children in Riga during the first year of life to evaluate the long-term findings. Fifty-nine children underwent surgical correction of AoCo at the age of 55 ± 61 days. The methods of surgical correction were anastomosis end-to-end (ETE) in 29% (n = 17), subclavian flap aortoplasty (SFA) in 64% (n = 38) and extended anastomosis end-to-end (EETE) in 7% (n = 4). Recoarctation developed in 15 patients (25%) with no difference between surgical techniques (P > 0.05). The recoarcation patients had left ventricle hypertrophy (left ventricle mass index (LVMi) 76 ± 19 g/m2.7) normalising after angioplasty (LVMi 42 ± 7 g/m2.7). Patients with recoarctation had a decreased pulsed wave (PW) Doppler systolic/diastolic ratio in abdominal aorta 2.3 ± 0.4 versus patientswithout recoarctation 5.3 ± 1.2 and the control group 6.3 ± 1.4 (P < 0.05). A high incidence of recoarctation exists in patients operated on for AoCo as small infants. Life-long surveillance is required to monitor and to intervene in a timely way. Supplementation of the echocardiographic protocol with an evaluation of PW Doppler flow pattern in abdominal aorta can provide additional information about the presence of obstruction.

Intrapulmonary Administration of Autologous Bone Marrow Derived Mononuclear Cells in Congenital Heart Disease Complicated by Pulmonary Arterial Hypertension: A Case Report

Pulmonary arterial hypertension (PAH) is a devastating, progressive disease with increasingly debilitating symptoms that leads to right heart failure and ultimately death if untreated.

Aortic coarctation repaired within the first year of life: an 11 year review

Aortic coarctation repaired within the first year of life: an 11 year review Introduction. Congenital heart diseases (CHD) affect 8-12 per 1000 live-born infants and it is one of the most common and serious congenital anomalies, aortic coarctation (AoCo) accounts for 6-10% of all the congenital heart diseases, and if not diagnosed early in life it can result in severe morbidity and mortality (3,9,13,11). Aim of the study. The aim of the study was to analyze the epidemiology of AoCo in newborn in Latvia, to evaluate a single centre 11 year experience with children who underwent AoCo repair in our institution within the first year of life, to define morbidity, mortality, risk factors, short and long term results. Materials and methods. The study was approved by the committee of ethics of the university hospital for children. Retrospective and prospective study/follow-up of all (74) patients younger than 12 months undergone native coarctation repair in our institution between January1, 2000 and December 31, 2010. Results. The prevalence of the coarctation of the aorta in Latvia was 3.43+/-1.2 per 10 000 life born infants. Seventy-four neonates and infants were analyzed: 44 boys (59%) and 30 girls (41%). Median age at the time of primary surgical correction was 47.3+/-58 days, medium weight 4.2+/-1.6kg. Prostoglandin E1 was used in 62%, inotropic stimulation in 20% of cases, assisted ventilation in 18%. According to the anatomy of the congenital heart disease patients constituted group I- the patients with simple coarctation with and without atrial septal defect (ASD) in 57%, group II -patients with coarctation and ventricular septal defect (VSD) 23%, group III- complex coarctation 20%. The infantile juxtaductal AoCo with isthmus hypoplasia was detected in 83%(n=62), postductal AoCo in 4%(n=3) and juxtaductal membrane in 12%(n=9), hypoplasia of the aortic arch in 16%(n=12). The techniques for primary repair included the resection with simple anastomosis end-to-end (ETE) in 26% (n=19), subclvian flap aortoplasty(SFA) in 65%(n=48) and extended anastomosis end-to-end (eETE) in 8%(n=6), primary balloon angioplasty in 1 case. There were 5 cases (6,8%) of early postoperative death and 9 cases of later death. Recoarctation occurred in 14 patients (23%), all underwent balloon angioplasty with no significant residual gradient, and there was no mortality or complications after reinterventions. Conclusions. The surgical correction of the AoCo remains gold standard for neonates and small infants. There were no statistically significant differences between the incidences of recoarctation dependent on the method of primary surgical correction. The incidence of recoarctation in cases of primary surgical correction early in life remains comparatively high. Baloon angioplasty is a method of choice in cases of recoarctation, it is safe and effective with low incidence of persistent coarctation. Mortality is most importantly influenced by preoperative status, the severity of associated anomalies, surgical outcomes, perioperative intensive treatment.

First Results of using Stem Cell Transplantation for Pediatric Patients in Case of Dilated Cardiomyopathy

First Results of using Stem Cell Transplantation for Pediatric Patients in Case of Dilated Cardiomyopathy Introduction. Dilated cardiomyopathy is a serious disorder of the myocardium in pediatric age. Conservative therapy is limited and lethal outcome observed in one third of patients within a year. Bone marrow derived progenitor cell transplantation is becoming a promising method of treatment in adult population and there is ground to believe there are perspectives in pediatric cardiology. Aim of the Study. We present the first results of bone marrow cell transplantation in case of dilated cardiomyopathy for six patients at age four months to seventeen years. Materials and methods. We did the six Bone marrow derived progenitor cell (BMCs) intramycardial transplantations. Five to 30 mililiters of bone marrow were aspirated from iliac bone. Seventeen to 90 million BMCs were isolated and as suspension of physiologic saline given to patients by intramyocardial punction. Every patient underwent repeated examination every two month. Results. Six months following bone marrow derived progenitor stem cell intramyocardial transplantation we observed increase of ejection fraction in 4 patients, decrease of dilatation echocardiographycally and cardiothoracic ratio (CTR) at chest x-ray; decrease of the stage of heart insufficiency clinically from class IV to I-II(NYHA). Conclusions. We see the intramyocardial administration of bone marrow cells proved to be technically feasible and safe, also the procedure does not need for expensive technical equipment. The bone marrow transplantation improves the patients clinical situation and physical measurements. Our method might be used for the stabilization of the patient to get the time for further treatment.