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.
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.
Transcatheter pulmonary valve implantation has been a well-known method for more than a decade, but there are still many challenging cases when a personalized solution is needed. We report a case of a 15-year-old female patient with tetralogy of Fallot, who underwent a surgical correction during infancy. Because of progressive pulmonary regurgitation, stenosis, and right ventricle dilatation, transcatheter pulmonary valve implantation in the native right ventricle outflow tract (RVOT) using Edwards SAPIEN valve was performed. A “landing zone” was created prior to the intervention of stenting the RVOT and the right pulmonary artery. The transcatheter approach for pulmonary valve replacement in a native RVOT is a reasonable alternative to the surgical approach.
AIM OF THE DEMONSTRATIONWe present a case of congenital heart rhythm disorders in an infant which carry serious risk of sudden death if left untreated. CASE REPORTThe child was sent to the hospital at the age of 21 days by the general practitioner due to bradycardia detected within a routine visit. The girl was born from the first pregnancy in urgent cesarean section due to fetal distress (umbilical cord wrapped around the neck) at 40 weeks of gestation, birth weight 3090g, height 54cm, Apgar score 7/9 and was discharged from the maternity hospital at the age of 4 days. The child received breastfeeding and parents did not have any complains about the newborn. The mother of the child is 21 years old and the father 23 years old, both completely healthy. The course of the pregnancy was uneventful. There were no cases of heart rhythm disorders or sudden death in the family history, no chronic illnesses in the family history. At the time of hospitalization (at the age of 21 days) the weight of the child was 3265g, bradycardia-heart rate 72 times per minute was detected, no heart murmurs, no hepatomegaly. Repeated electrocardiograms (ECG) showed first to second degree second type ( Mobiz II) atrio-ventricular block and prolonged QT interval 0,52, cQT 0,54 as well as complete right and left anterior bundle branch block. Echocardiography was completely normal with LVDd 22mm, fractional shortening 37% and ejection fraction 61%. 24 hour ECG monitoring reveled first to second degree second type ( Mobiz II) atrio-ventricular block and prolonged QT interval and repeated paroxysms of ventricular tachycardia with the ventricular rate up to 251 beats per minute ( the longest one 49 seconds). No changes were found in thoracic x-ray, neurosonography and abdominal ultrasound. There were neither signs of Summary Long QT syndrome (LQTS) is a disorder of myocardial repolarization characterized by prolonged QT interval on ECG with prevalence close to 1/3000-1/5000. LQTS is characterized by the occurrence of syncopal episodes due to torsades de pointes ventricular tachycardia (VT) and by a high risk for sudden cardiac death among untreated patients (1, 2, 3). In 12% of patients with LQTS, sudden death is the first manifestation of the disease and only in 4% this happens in the first year of life (2). There is consensus that all symptomatic children with LQTS should be treated with β-blockers which are effective in preventing cardiac events and reducing mortality in 70%, but do not protect patients from sudden death completely (1,2,3,4). The prognosis is poor in untreated patients with annual mortality 20% and 10 year mortality up to 50% (1, 2). Here we present a case of relatively rare congenital heart rhythm disorders in an infant which required immediate treatment.
SummaryCor triatriatum sinister is a rare congenital cardiac anomaly that has been identified in 0.1% of children with congenital heart disease. It is defined as a fibromuscular membrane that divides the left atrium into two chambers: a superior (proximal) that in most cases receives drainage from the pulmonary veins and an inferior (distal) chamber that communicates with the mitral valve and the left atrium. Cor triatriatum sinister can be an isolated lesion (approximately 25% of cases), but in many cases it is associated with other congenital cardiovascular anomalies, the most common one being – atrial septal defect(3). Symptoms in patients with cor triatriatum sinister are related to obstruction of pulmonary venous drainage, pressure loading of the right side of the heart and congestive cardiac failure. Depending on the severity of the obstruction and presence of associated cardiac anomalies it can be diagnosed at any age. Diagnosis is usually achieved by echocardiography in early infancy. Elective treatment method is surgical excision of the membrane. Here we present a pediatric patient (4 months old) presenting in cardiogenic shock with a successful correction of isolated cor triatriatum sinister. To confirm diagnosis and success of surgical repair, transthoracic and transesophageal echocardiography were used.
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