Complex Cardiovascular Anomalies Research Articles

Surgical management of congenital tracheal stenosis associated with complex cardiovascular anomalies.

Although surgical outcomes have improved in patients with congenital tracheal stenosis (CTS), the management of such patients with complex cardiovascular anomalies (CVAs) remains a challenge. This study aimed to clarify the clinical features of this combination. Medical records of 14 patients with complex CVAs who had undergone slide tracheoplasty for CTS between May 2016 and February 2022 were retrospectively reviewed. Complex CVAs were defined as CVAs without left pulmonary artery sling and simple cardiac shunts. Data collected included age and body weight at tracheal reconstruction, preoperative respiratory support, surgery details, and mortality. The median age and body weight at tracheal reconstruction were 3.5 (range, 1-17)months and 4.7 (range, 2.3-8.2)kg, respectively. Simultaneous repair of CTS and CVAs was performed in eight patients and staged repair in six patients. Patients who underwent simultaneous repair required preoperative respiratory support more frequently than those who underwent staged repair (8/8 [100%] vs 2/6 [33.3%]; P = 0.015). No mortality occurred. Patients with complex CVAs often require tracheal reconstruction in early infancy. Surgical management strategy for this combination was generally decided on the basis of the severity of respiratory symptoms due to CTS.

Morphological features of complex congenital cardiovascular anomalies in fetuses: as evaluated by cast models

Accurate prenatal diagnosis of complex congenital cardiovascular anomalies, vascular ones in particular, is still challenging. A fetal cardiovascular cast model can provide a copy of the cardiac chambers and great vessels with normal or pathological structures. This study was aimed to demonstrate three-dimensional anatomy of complex congenital cardiovascular anomalies in fetuses by means of corrosion casting. Twenty fetuses with prenatal-ultrasound-diagnosed complex cardiovascular anomalies were enrolled in this study (19 to 35 gestational weeks). Fetal cardiovascular cast models were made by a corrosion casting technique. The specimens were injected with casting material via the umbilical vein, and then immersed in strong acid after casting fluid was solidified, to disclose the geometries of cardiovascular cavities. Nineteen cast models were successfully made from 20 specimens. The casts distinctly showed the morphological malformations and spatial relationship between cardiac chambers and great vessels. One hundred and eleven abnormalities were revealed by casting in the 19 specimens, including 34 abnormalities located in the cardiac chambers (3, 4 and 27 anomalies in the atria, atrioventricular valves and ventricles, respectively), and 77 in the great vessels (28, 20, 24 and 5 anomalies in the aorta and its branches, the pulmonary artery, the ductus arteriosus and the major veins, respectively). Corrosion casting can display three-dimensional anatomy of fetal complex cardiovascular anomalies. This improves our understanding of related pathomorphology and prenatal diagnosis.

Morfologia kliniczna przegrody serca i jej wad rozwojowych

Malformations of the cardiac septum associated with intracardiac shunting constitute the most common group of congenital heart defects in the human population. They can be isolated or exist as a part of more complex cardiovascular anomalies. Importance of septal defects implicates a need for precise description of anatomy and embryogenesis of interatrial, atrioventricular and interventricular septums in a way useful for diagnosis and therapy. This paper presents morphology of the cardiac septum consistent with current international bibliography. True atrial septal defects are separated from other forms of interatrial communication. Common features of all atrioventricular septal defects are described. Morphological criteria independent on numerous existing classifications are presented as a base for accurate description of ventricular septal defects. In the era of rapidly developing prenatal cardiology, paediatric cardiology and cardiac surgery implicating effective treatment of intracardiac shunts, the knowledge about anatomy of cardiac septal malformations is crucial for specialist in various fields of medicine. Unified communication code assures appropriate cooperation of physicians involved in treatment of patients in every period of their life.

823 Epidemiological Characteristics of Down Syndrome in the Region of Western Herzegovina

During the 15 years period (1994-2008), 46 children with Down syndrome (DS) (31 boys and 15 girls) have been registered at the Neonatology Department of the University Hospital Mostar (Bosnia and Herzegovina). The average prevelence of DS for the region of Western Herzegovina (WH) (217 944 inhabitants) is 1,5 per 1,000 newborns in that period. DS has been proved by means citogenetic analyses in 38 children. In 36 there was a regular type of trisomy 21 (94.7%) and in 2 the translocation type of DS was found. 33 parental couples (71.7%) were over 35 years of age. Early amniocentesis due to the parent's age was made in two cases only, with positive dignosis. Usually, parents are refusing prenatal diagnosis due to religous reasons. All children with DS have had five or more easily recognized minor malformations present in variable combinations. 28/46 had major malformations and three children have had mild forms of conatal hypothyreosis. Seven children (15.2%) died in neonatal or early infant period due to sepsis and complex cardiovascular anomalies. Considering evidently present major malformations and accompaning complications, DS remains very important issue for routine pediatric clinical practice.

Coronary microcatheter mapping of coronary arteries during radiofrequency ablation in children

Radiofrequency catheter ablation (RFCA) is a safe and effective treatment option for children with refractory supraventricular tachycardias. RFCA of right-sided accessory pathways (APs) continues to be challenging, resulting in lower success rates and higher recurrence rates. One of the reasons for a prolonged or failed procedure is mapping error and changes in cardiac anatomy. The right coronary artery (RCA) runs along the ventricular aspect of the right epicardial atrioventricular groove, and RCA mapping had been reported facilitating difficult right-sided AP RFCA in the pediatric population. We reported that microcatheter-assisted mapping during RFCA can improve localization of right-sided and some left-sided APs particularly in children with congenital heart disease. This technique was used in seven patients with a mean age of 15.3+/-1.6 years and a mean weight of 52.6+/-6.5 kg. Five patients (71%) had manifest preexcitation on surface electrocardiogram, and two patients had a concealed AP. In two of them, there were single ventricle anatomies. In the other two patients, venous access problem to the heart and enormous cardiac hypertrophy were the major problems. In the remaining three patients with normal cardiac anatomy, RCA mapping was used because a standard procedure was not successful. Previous RFCA ablation was not successful in two patients. Either a 150-cm eight-electrode pair (2-6-2 mm, 2.5 French) or a four-electrode pair (2-6-2 mm, 2.3 French) microcatheter (2.3 French octapolar microcatheter) was used. Average time of the multielectrode RCA mapping was 42+/-14.5 min. All eight (100%) APs were successfully ablated. No complications occurred. Microcatheter-assisted coronary artery mapping during RFCA is a useful adjunctive mapping technique in children with difficult to map right-sided APs and patients with APs who has complex cardiovascular anomalies.

An approach to dissecting the congenitally malformed heart in the forensic autopsy: the value of sequential segmental analysis.

The demonstration of congenital heart disease at autopsy necessitates the careful preservation and examination of the heart, the vessels, and their connections. Techniques preserving these connections and using a reproducible and systematic approach are preferred. The Rokitansky method of organ block dissection, in combination with a system of heart examination termed sequential segmental analysis, provides such an approach. This study is based on the examination of heart specimens accessioned into the Frank E. Sherman, M.D., and Cora C. Lenox, M.D., Heart Museum (containing approximately 2400 specimens) of the Pathology Department, Children's Hospital of Pittsburgh. Specimens received in consultation during a 25-year period from hospitals and coroners'/medical examiners' offices were examined, and the corresponding reports were reviewed. Of 46 total heart specimens examined (1975-1999), 29 (63%) were dissected properly or left intact for dissection at Children's Hospital of Pittsburgh, and 17 (37%) were incorrectly dissected for the demonstration of congenital heart disease. Of these 17 cases, 11 (24%) displayed dissection errors, which did not hinder a complete diagnosis, 3 cases (6.5%) had errors that enabled only an incomplete diagnosis, and in 3 cases (6.5%), no diagnosis of congenital heart disease could be made. Dissection mistakes and means of avoiding them are discussed. Review of medical and family history, external and internal examination, and a reproducible and sequential method of examining the heart and its connections enables documentation of even the most complex cardiovascular anomalies.

The Italian Multicentric Study on Epidemiology of Congenital Heart Disease: first step of the analysis. Working Party of the Italian Society of Pediatric Cardiology.

We present the aims, methodology and initial results from the Italian Multicentric Study for the registration and follow-up of congenital heart disease. The general aims are to measure the prevalence of congenital heart disease in different geographic areas of Italy, and to assess the survival and outcome of affected babies. During the years 1992 and 1993, eighteen centers for Pediatric Cardiology spread all over the Country enrolled 1445 new babies with congenital cardiac malformations from a population of 341,647 surveyed livebirths. The new cases were registered using the same methodologic criteria of the EUROCAT study in order to evaluate differences and/or similarities between the studies. The prevalence varied between 1.8% and 8.1%; the average being 4.6%. The large range in prevalence is presumed to be related to different customs and hierarchies in flow and referral of patients. We provide total prevalence of individual lesions, and distribution of sentinel cardiac anomalies, in the Italian study and compare them with EUROCAT. Isolated ventricular septal defect is the most common lesion (39%); followed by atrial septal defect (7.5%); pulmonary valvar stenosis (7.3%); atrioventricular septal defects (5.4%); patency of the arterial duct (3.8%); complete transposition (3.7%); tetralogy of Fallot (3.3%); aortic coarctation (2.4%); aortic valvar stenosis (2.2%); and left heart hypoplasia (1.8%). The echographic stratification of ventricular and atrial septal defects, by location and size, was in keeping with the findings of the EUROCAT study. Because of the recent widespread availability of color-Doppler techniques, the stratification of aortic and pulmonary valvar stenosis was an innovative approach in our study. Among the complex cardiovascular anomalies, double inlet ventricle and pulmonary atresia had a proportion of about 2% each; with double outlet right ventricle, common arterial trunk, Ebstein's malformation, tricuspid atresia, interrupted aortic arch and totally anomalous pulmonary venous connection having a proportion ranging from 0.5 to 0.8%. We discuss clinical features, such as frequency of extracardiac anomalies and familial aggregation of congenital heart disease, in comparison with the EUROCAT data.

Anomalous looping, atrioventricular cushion dysplasia, and unilateral ventricular hypoplasia in the mouse embryos with right isomerism induced by retinoic acid.

Visceroatrial heterotaxy syndrome is characterized by abnormality of visceral laterality and complex cardiovascular anomalies usually involving both the outflow and inflow tract. Morishima et al. (1995) showed that mouse embryos treated with all-trans retinoic acid at embryonic day 6.5 (primitive streak stage) induces this syndrome. To investigate the morphogenetic process of visceroatrial heterotaxy syndrome, we examined retinoic acid-treated mouse embryos at embryonic days 9-15 using scanning electron microscopy. The sinoatrial connection was first distinguished for the determination of situs as early as at embryonic day 10.5. Normal visceroatrial situs was found in 57% of all treated embryos, and the rest had abnormal situs, in which right isomerism was found in 81%. In the right-isomeric mouse, the cardiac morphology was characterized by abnormal looping together with dysplasia of the inflow and outflow tract cushion; that is, the primitive right ventricle was usually deviated cranially to various degrees, the atrioventricular cushion appeared trilobed in a half of them, and unilateral ventricular hypoplasia was noted in about one-third of them after embryonic day 14.5. An anomalous relation between the atrioventricular cushions and the interventricular septum appeared to have caused a restrictive inflow to the unilateral ventricle, leading to ventricular chamber hypoplasia on the ipsilateral side. Thus, we clarified that retinoic-acid treatment at the primitive streak stage disturbed cardiac looping and formation of atrioventricular cushion development, which secondarily influenced ventricular chamber development.

Surgical palliation of cardiac malformations associated with right isomerism.

Between 1985 and 1993, palliative surgery was performed on 13 pediatric patients who had complex cardiovascular anomalies associated with right isomerism. The patients included two neonates, ten infants, and one child who were divided into two groups according to whether or not a total anomalous pulmonary venous connection (TAPVC) was present. Group 1 consisted of six patients with TAPVC and group 2 consisted of seven patients without TAPVC. In group 1, the surgical procedures involved TAPVC repair alone in two patients, combined TAPVC repair with a modified Blalock-Taussig shunt in two, combined TAPVC repair with pulmonary artery banding in one, and a modified Blalock-Taussig shunt alone in one. There were five hospital deaths and one late death in this group: pulmonary venous obstruction in two patients, perioperative myocardial failure in the two neonates, and congestive heart failure caused by increased pulmonary blood flow in two patients. In group 2, all the patients underwent systemic-pulmonary artery shunts, and there was one hospital death and three late deaths, the causes of which were unknown in two patients, and shunt failure and pneumonia in one patient each. These results suggest that surgical palliation for right isomerism produces poor results in young infants with obstructed TAPVC. Thus, we conclude that TAPVC repair should be performed without delay if pulmonary venous obstruction has been diagnosed clinically. Resolving pulmonary venous obstruction without cardiopulmonary bypass (CPB) may be preferable for infants, considering their difficult management. The systemic-pulmonary artery shunt should be of the low-calibrated type, especially if common atrioventricular valve regurgitation exists. If infants survive the surgery, they must be carefully followed up for a long period due to the risk of sudden death or infection.

Right and Left Isomerism: The Cardiac Surgeon's View

We have treated by palliative or corrective surgery 12 patients with the complex cardiovascular anomalies associated with right isomerism (asplenia syndrome) or left isomerism (polysplenia syndrome). A systemic-pulmonary artery shunt was performed in 4 patients, with 2 early deaths. Anatomical intracardiac repair was successfully done in 2 patients, while an orthoterminal repair using a modified Fontan procedure was attempted in 5 patients, only 1 of whom survived. A 1-year-old girl with diffuse pulmonary arteriovenous fistulas associated with left isomerism was not considered a good candidate for operation after an exploratory sternotomy. The surgical options and technical problems related to the complex intracardiac morphology of these developmental syndromes are presented.

Situs inversus totalis associated with complex cardiovascular anomalies

Two cases of situs inversus totalis complicated by complex cardiovascular anomalies are presented. In Case 1 the associated cardiovascular abnormalities consisted of an anteriorly located aorta and systemic ventricle, ventricular septal defect, pulmonic stenosis, and partial anomalous pulmonary venous drainage, while in Case 2 a ventricular septal defect and an absent inferior vena cava with functional replacement by the azygous vein were diagnosed. Cardiac catheterization and angiocardiography were utilized in order to obtain accurate preoperative anatomic and functional data. Embryologic aspects and the various cardiac anomalies associated with situs inversus totalis are discussed.