Non-cardiac Congenital Anomalies Research Articles

Surgical Correction of Common Atrium without Noncardiac Congenital Anomalies

Common atrium (CA) is a rare congenital heart defect. We reviewed our experience of surgical treatment of CA and summarize the clinical features of CA and the key techniques for surgical correction. Between August 1984 and August 2010, 37 consecutive cases of CA underwent corrective surgery. There were no clinical findings of Down, asplenia-polysplenia or Ellis-van Creveld syndromes in all cases. Mitral valvuloplasty was performed in 34 cases, and tricuspid valvuloplasty in 20 cases. Complete closure of a mitral cleft was required in 32 cases. All new atrial septa were reconstructed using patches. Follow-up period ranged from 1 to 20 years. There were no hospital deaths or conduction system block. After surgery, mild mitral insufficiency was observed in only one case. Two cases had moderate-severe mitral insufficiency at postoperative years 1 and 3, respectively, that required mitral valve replacements. One patient died of low cardiac output syndrome after reoperation. Long-term survival after surgical correction of CA is good. Routine closure of a cleft in the mitral valve is very important for successful surgery.

Gastrointestinal Complications After Stage I Norwood Versus Hybrid Procedures

Patients undergoing palliation for hypoplastic left heart syndrome are at risk for gastrointestinal complications including necrotizing enterocolitis, gastroesophageal reflux, and intolerance to oral feeding. Few data exist comparing these outcomes between patients undergoing the Norwood and hybrid procedures. Forty-three patients with hypoplastic left heart syndrome or variants underwent 50 Norwood (n = 34) or hybrid (n = 16) procedures (2004 to 2011). Routine preoperative assessments included flexible fiberoptic laryngoscopy and oral feeding evaluations. Examinations were repeated postoperatively with modified barium swallows. A retrospective review was conducted to examine the incidence, causes, and consequences of gastrointestinal complications. In 18 of 50 cases (38%), patients were tolerating full oral feeding at discharge; abnormal preoperative feeding evaluation predicted the inability to feed orally (p < 0.002, positive predictive value 100%). Hybrid and Norwood patients had a similar incidence of vocal fold palsy (20.0% versus 34.5%, p = 0.3), and inability to feed orally at discharge (67.5% versus 56.3%, p = not significant). Both groups had a high incidence. Postoperative abnormalities were common, namely, abnormal oral feeding evaluations (70.7%) or modified barium swallows (81.8%). Predictors of abnormal postoperative examinations included noncardiac congenital anomalies (p = 0.08), preoperative mechanical ventilation (p = 0.01), and younger age (p = 0.01). Grade IIA/IIB necrotizing enterocolitis was more common among patients having hybrid procedures (26.7%, versus 2.9%, p = 0.01). Gastrointestinal complications are common after initial palliation of patients with hypoplastic left heart syndrome. Preoperative and perioperative factors play a significant role. Despite a more "limited" intervention, patients undergoing hybrid procedures remain at high risk for feeding issues, commonly leading to a prolonged hospital course. Close attention and proactive management are essential to optimize the nutritional status in these patients.

The Effect of Late Preterm Birth on Mortality of Infants with Hypoplastic Left Heart Syndrome

We evaluated the effect of late preterm (34 to 36 weeks' gestation) delivery on hospital mortality of infants with hypoplastic left heart syndrome (HLHS). Retrospective review of records of infants born at or after 34 weeks with no other lethal anomalies, cared for in a single tertiary perinatal center between 2002 and 2009. Factors associated with death prior to discharge from the hospital were ascertained using univariate and multivariate analyses. Of the 243 infants with HLHS, 35 were late preterm and 208 were ≥37 weeks (term). Using logistic regression analysis, late preterm delivery (odds ratio [OR] 2.95; 95% confidence interval [CI] 1.35 to 6.45), the presence of other major cardiac defects (OR 3.76; 95% CI 1.31 to 10.81), and the presence of noncardiac congenital anomalies (OR 6.13; 95% CI 1.43 to 26.22) were independently associated with hospital death. Late preterm birth of infants with HLHS was independently associated with an increased risk of hospital death compared with those delivered at term.

Hospital Mortality of Late Preterm Infants with Hypoplastic Left Heart Syndrome

Aim: We evaluated the effect of late preterm (born 34-36 weeks) delivery on hospital mortality of infants with hypoplastic left heart syndrome (HLHS).Methods: Retrospective review of records of infants born at or after 34 weeks with no lethal anomalies, cared for in a single tertiary perinatal center between 2002 and 2009. Factors associated with death prior to discharge from the hospital were ascertained using univariate and multivariate analyses. Mode of delivery, place of birth, presence of other cardiac and non-cardiac congenital anomalies, late preterm birth, intrauterine growth restriction, multiple birth, race and gender were the variables retained in the forward multivariate logistic regression model.Results: Of the 243 infants with HLHS, 35 were late preterm and 208 were ≥37 weeks (term). Both groups had similar total duration of hospitalization for survivors and non-survivors. Most of these infants (94% of the late preterm group and 95% of the term group) underwent palliative cardiac surgery. However, hospital mortality following these surgical procedures was significantly higher in the late preterm (42% vs 17%, p= 0.002). Using logistic regression analysis, (OR; 95% CI) late preterm delivery (2.95; 1.35 - 6.45), the presence of other major CHD (3.76; 1.31-10.81) and the presence of non-cardiac congenital anomalies (6.13; 1.43 - 26.22) were independently associated with hospital death.Conclusions: In a population of infants with HLHS who were considered to be candidates for surgical repair, late preterm birth was independently associated with increased risk of hospital death compared to those delivered at more mature gestational ages.

Left ventricular outflow tract obstruction: should cardiac screening be offered to first-degree relatives?

ObjectivesTo determine whether offering cardiac screening to relatives of patients with left ventricular outflow tract obstructions (LVOTOs) would be justified.BackgroundLVOTOs have been recognised as a group of congenital heart diseases...

Perioperative management of low birth weight infants for open‐heart surgery

Infants of birth weight ≤2500 g are termed low birth weight (LBW). These children often have considerable morbidity from prematurity and intra-uterine growth restriction. Additionally, LBW infants have increased risk for cardiac and noncardiac congenital anomalies and may require surgery. Primary rather than palliative surgical repair of cardiac lesions has been preferred in recent years. However, LBW remains a risk factor for increased mortality and morbidity after open-heart surgery (OHS). There is a paucity of information about the anesthetic challenges presented by LBW infants undergoing OHS. This review summarizes the perioperative issues of relevance to anesthesiologists who manage these high-risk patients. Emphasis is placed on management concerns that are unique to LBW infants. Retrospective data from the authors' institution are provided for those aspects of anesthetic care that lack published studies. Successful outcome often requires substantial hospital resources and collaborative multi-disciplinary effort.

Echocardiographic findings in children with surgically correctable non-cardiac congenital anomalies

Congenital cardiac anomalies may co-exist with non-cardiac congenital malformations and, for those requiring surgical correction, there can be an anaesthetic risk. To estimate the burden of congenital heart disease (CHD) in children with surgically correctable non-cardiac congenital malformations. Records of 120 children aged between 1 week and 11 years [mean (SD) 0.6 (1.5) years] undergoing corrective surgery for non-cardiac congenital malformations were examined. Results of clinical cardiac examination and surgical and echocardiographic findings were analysed. Cleft lip or palate was the commonest surgical anomaly (46, 38.3%), followed by ano-rectal malformation (32, 26.7%). Forty-two children (35.0%) had an abnormal echocardiographic scan. A cardiac abnormality was detected clinically and confirmed by echocardiography in ten patients (8.3%), which represents 24% of all the cardiac abnormalities. The commonest cardiac anomalies were atrial septal defect (ASD) (30 children, 25%), followed by ventricular septal defect (VSD) (seven children, 5.8%). The frequency of ASD was highest in children with a cleft lip or palate (14/46, 30.4%). Over one-third of patients undergoing surgical correction of congenital malformations have co-existing CHD. Echocardiography is important for pre-surgical evaluation. No association between type of CHD and specific non-cardiac congenital malformations was found.

Anesthesia for non-cardiac surgery in children with congenital heart disease

It has been estimated that 0.8% of children born in the United States will be diagnosed to have some form of congenital heart disease. (CHD) Many of these children will require surgery for noncardiac conditions that are commonly encountered during childhood (e.g. inguinal hernia, dental caries, or chronic tonsillitis). Moreover, many children with CHD have other associated noncardiac (e.g. orthopedic or genitourinary) congenital anomalies that require surgical repair. Many of these procedures are performed in general hospitals by noncardiac anesthesiologists. It is therefore essential that the anesthesiologist be knowledgeable, not only of the basics of pediatric anesthesia, but also of the pathophysiology of the cardiac lesions. Knowledge of the physiological consequences of shunting or obstruction is essential to understanding the hemodynamic consequences of any congenital heart deformity. Application of these physiologic concepts permits the anesthesiologist to design a rational management plan for the child with CHD.

Ten-year survival of Down syndrome births

To determine the survival status of children with Down syndrome (DS), and to document factors influencing survival. Follow-up study of cases identified from the Dublin European Register of Congenital Anomalies and Twins (EUROCAT) Register. Follow-up was attempted for each case until death or 1992 or until the date last known to be alive. Eastern Health Board, Dublin. In all, 389 DS children, born between 1 January 1980 and 31 December 1989 were followed up. Survival rates of 88% at one year and 82% at 10 years were found. There was a non-significant improvement in survival between the cohort born in 1980-1984 and that born in 1985-1989. Congenital heart defects reduced survival to 72% and complete atrio-ventricular canal defects (CAVD) had the poorest prognosis (58% survival at 10 years). Cases with CAVD showed a trend towards improved survival when surgically treated. Maternal age mother's county of residence, sex of infant, season of birth and presence of additional non-cardiac congenital anomalies had no impact on survival. Four out of five DS children now survive at least 10 years. Adequate educational and health service provision needs to be made for them, especially those with congenital heart defects. The need for studies which compare survival and quality of life in DS children with CAVD who undergo cardiac surgery versus those who do not, taking account of various selection factors, is identified.

Anomalies of pulmonary venous return.

AbstractTotal anomalous pulmonary venous return is a rare but life‐threatening congenital heart lesion. We present our experience with 48 consecutive infants operated on from 1977 to 1983. Improved results are due to earlier diagnosis, improved surgical technique, and meticulous postoperative care. Since 1980 some patients with this lesion were submitted to surgery on the basis of two‐dimensional echocardiography, thus avoiding cardiac catheterization and angiocardiography. The operative procedure should achieve a large unobstructed anastomosis between the pulmonary veins and the left atrium. Details of operative techniques currently used in our unit are discussed for each anatomic type of total anomalous pulmonary venous drainage. Although the results are improving, the overall mortality rate (hospital and late) remains around 30% in most series. Very young age (neonates), poor preoperative condition, and presence of associated cardiac and noncardiac congenital anomalies increase the risk. There were 12 hospital and 2 late deaths in our series of 48 infants (29%). Detailed analysis of the causes of death is presented.

Surgical management of vascular ring.

Between 1968 and 1980, 51 children had an operation for various forms of vascular ring. Additional cardiac malformations were present in five patients, and six had noncardiac congenital anomalies. Although symptoms started within the first month of life in 39 infants, only 16 came to operation under three months of age, and a delay of more than six months occurred in 15. Stridor, often life-threatening, and recurrent infections were the most common symptoms, but dysphagia was also important. The reasons for delay in diagnosis are discussed. Barium swallow provided the diagnosis in 44 patients and suggested it in a further four patients. Innominate artery compression of the trachea was not diagnosed by barium swallow. Operations of various types were performed. Accuracy in diagnosis is important because not all patients could be treated through the classic left thoracotomy. Severe tracheomalacia was responsible for the only two deaths in the series; one of these had tracheostomy performed in the referring hospital, the second child also had Fallot's tetralogy. The large majority of the patients have done well, 76% being asymptomatic at follow-up. Minimal to moderate stridor persists among the remainder to the present time.

Patent Ductus Arteriosus: An Analysis of 515 Surgically Proved Cases

SUMMARY AND CONCLUSIONS During the years 1938 to 1958, 435 patients with isolated patent ductus arteriosus and 80 patients with additional cardiac lesions have been operated on at the University of Minnesota Hospitals. The vast majority of patients over one year of age were described as having a machinery-like murmur and a thrill maximal in the second and third left intercostal spaces. During infancy, however, the clinical picture was frequently indistinguishable from that of a ventricular septal defect. Accordingly, special diagnostic procedures were more frequently employed during infancy. Cardiac catheterization and retrograde aortograms were equally successful as a means of obtaining correct diagnoses. Mean pulmonary artery pressures correlated reasonably well with the type of murmur and electrocardiographic patterns. Ligation of the ductus was employed in only 7 per cent of the cases. In the remainder of the cases the ductus was transected. There were ten deaths (2.3 per cent) mortality rate in isolated patent ductus. Nine patients had definite episodes of subacute bacterial endocarditis prior to surgery. One of them died as a result of the tearing of the fragile ductus during surgery. The remaining cases underwent uneventful surgical corrections. Pregnancy, a previous history of rheumatic fever, maternal rubella, or the presence of non-cardiac congenital anomalies did not significantly affect the surgical outcome. Even more surprising was the fact that the operation during infancy did not adversely affect the surgical mortality. Only three patients were thought to have excessive postoperative bleeding in spite of a high incidence of abnormal blood coagulation tests. None of the 61 patients with frequent epistaxis, menorrhagia, or hemoptysis had excessive postoperative bleeding. The operative mortality rate for males was double that for females. The trend at this center has been toward earlier surgery, usually as soon as a definite diagnosis has been established. Patients who are diagnosed in early infancy and who are otherwise normal may be watched for a few months.