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PubMed1992

Major surgical intervention during extracorporeal membrane oxygenation.

Of 135 patients treated with extracorporeal membrane oxygenation (ECMO) between January 1987 and December 1989, 19 (14.0%) patients underwent surgical procedures while on ECMO. Thirteen (68%) patients had operations related to hemorrhage, including cannula site (6), mediastinal (1), hemoperitoneum (3), and hemothorax (3). Six of 13 patients required repetitive operations for bleeding; 4 of 6 died. Six (35%) patients had operations for congenital pathology including patent ductus (PDA) ligation (2), repair of transposition of the great vessels (2), repair of coarctation (1), and repair of congenital diaphragmatic hernia (3). One patient had multiple simultaneous procedures performed. Of these 6 patients, 4 were decannulated immediately and 2 were decannulated within 28 hours following surgery without any bleeding complications. Fifteen of 19 patients were operated on in the neonatal intensive care unit. The 4 remaining patients required transport on ECMO to the surgical suite. Thirteen of the 19 patients requiring surgical intervention on ECMO survived. In the 13 survivors, the mean time to decannulation postoperative was 45 hours, and in those that died it was 90 hours. Our experience suggests that surgical intervention while on ECMO is technically feasible with the best results achieved when rapid discontinuation of ECMO can be accomplished postoperatively. Due to this fact major surgical intervention should be postponed if possible until near the conclusion of the ECMO therapy.

Algorithms↗
PubMed1994

Predictors of postoperative outcome after general surgical procedures in patients with congenital heart disease.

This study was undertaken to evaluate factors predictive of postoperative outcome after general surgical (GS) procedures in patients with congenital heart disease (CHD). All patients with a diagnosis of CHD who underwent a GS procedure under general anesthesia during a consecutive 10-year period were considered eligible for the study The congenital heart defect was classified as either simple (ASD, VSD, PDA) or complex (endocardial cushion defects, transposition of the great vessels, tetralogy of Fallot), and the GS procedure as either major (intraperitoneal, intrathoracic, or vascular reconstructive) or minor (inguinal herniorrhaphy, vascular access). The overall mortality rate for the patient population was 12% (27 deaths among 226 procedures), minor procedures being associated with a 3% mortality rate (2 of 70 procedures), and major procedures with a 16% mortality rate (25 of 156 procedures). Incremental risk factors for mortality included a preoperative American Society of Anesthesiologists' (ASA) physical status class of IV or higher (P = .0003), a preoperative in-hospital stay of 10 or more days (P = .004), birth at a tertiary care center (P = .04), and emergency operations (P = .05). In the subgroup of patients less than 6 months old, weight of less than 2.4 kg at the time of surgery and a 1-minute Apgar score of less than 4 were additional independent risk factors (P = .04 and .01, respectively). By logistic analysis, previous corrective cardiac procedures, whether complete or palliative, did not significantly alter the postoperative outcome. The authors conclude that physiologically well-compensated patients with CHD can undergo elective operations at a low operative risk; however, poorly compensated patients undergoing urgent or emergent operations are at high risk.(ABSTRACT TRUNCATED AT 250 WORDS)

Anesthesia, General↗
PubMed1986

An anatomical study of the patterns of the coronary arteries and sinus nodal artery in complete transposition.

The origin and course of the main coronary arteries and the sinus nodal artery are relevant to both intra-atrial and arterial relocation operations for complete transposition (the combination of concordant atrioventricular and discordant ventriculo-arterial connexions). The patterns of the main coronary arteries in 148 hearts with complete transposition have been documented together with the patterns of the sinus nodal artery in 105 of these. The terminology which has been used to describe these arteries has been explained. The findings illustrate the wide variation which exists in the origin and course of all these arteries in complete transposition and indicate areas where they are most at risk.

Arteries↗
PubMed1981

The heart and diaphragm: target organs in the neonatal death induced by nitrofen (2,4-dichlorophenyl-p-nitrophenyl ether).

This paper examines the effects of in utero exposure to 2,4-dichlorophenyl-p-nitrophenyl ether (nitrofen) on the viability of neonatal Long-Evans rats. Oral administration of this herbicide on days 8-18 of gestation reduced neonatal survival and birth weight. Day 11 of gestation was the most sensitive day for induction of neonatal mortality; 116 mg/kg to the dam on this day was the LD50 for the neonate. An increased incidence of hydronephrosis was observed in 35-day survivors. This increase was dose-related in animals exposed on day 11 of gestation. Fetuses exposed on day 11 and examined at term had reduced weights, delayed skeletal ossification, and an increased frequency of hydronephrosis and diaphragmatic hernias. While nitrofen did cause a high incidence of hydronephrosis, BUN or creatinine levels in 4-h neonates were not elevated. Detailed examination of the hearts of term fetuses revealed cardiac malformations classified as ventricular septal defect, double outlet right ventricle, and transposition of the great vessels. We conclude from these studies that the heart and the diaphragm are the target organs in nitrofen-induced neonatal death.

Abnormalities, Drug-Induced↗
PubMedDate not supplied

Magnetic resonance imaging in congenital heart disease of newborns: preliminary results in 23 patients.

Until now little attention has been paid to the potential of MR imaging in congenital heart disease of the newborn. ECG-gated MRI was therefore performed at 0.5 tesla in 23 newborns (mean age 7.5 days) with suspected congenital heart disease. Two newborns were controlled after surgery (switch, Blalock-Taussig procedure). All had undergone prior evaluation by two-dimensional Doppler echocardiography (2-D DE). MR imaging was of a satisfying quality in all but one newborn. The aim of this study was to assess complementary information provided by MRI in comparison to 2-D DE. Pre-operatively MRI missed some abnormalities shown by 2-D DE: one coartaction, one ductus arteriosus and one pulmonary atresia. MRI demonstrated lesions that echocardiography had either failed to visualize or found inconclusive, including double aortic arch (one patient), muscular ventricular septum defect (two patients) and severe ductus arteriosus (one patient). In one of the two patients with a ventricular septum defect, angiography was avoided and in the other patient it merely confirmed the MRI results. Post-operatively, MRI demonstrated information complementary to that obtained from to 2-D DE: (1) clearly visualizing the reinsertion of the coronary arteries in the 'switched' transposition of the great vessels, (2) appreciating the diameter and patency of the palliative shunt in the Blalock-Taussig procedure.

Angiography↗
PubMed2004

Assessment of intracardiac anomalies in two adults with Ivemark's syndrome.

Ivemark's syndrome consists of intracardiac anomalies, abnormal lobation of the lungs, and abdominal heterotaxy. A frequent intracardiac anomaly seen in Ivemark's syndrome is a common atrium, which is associated with left-to-right shunting. The increased blood flow and resistance within the pulmonary vasculature creates pulmonary hypertension and eventual reversal of the shunt physiology. In the absence of additional cyanotic malformations, survival into adulthood without prior surgical septation of the common atrium depends on the extent of pulmonary hypertension and intracardiac right-to-left shunting. We present two patients with a common atrium in the setting of Ivemark's syndrome who survived into adulthood without prior operation. Two-dimensional echocardiography assessed their intracardiac structures. One patient had right atrial isomerism manifested by asplenia and a common atrium, into which the hepatic veins drained directly, and the other patient had left atrial isomerism manifested by polysplenia, a common atrium, and a ventricular septal defect with a single atrioventricular valve. Neither patient had additional cyanotic malformations, including obstruction to pulmonary venous return, transposition of the great vessels, or pulmonic valve stenosis. The 2-dimensional echocardiogram guides the clinician to refer patients for surgical septation of the common atrium before the right-to-left shunt physiology predominates. The medical and surgical treatment of these patients is discussed.

Abnormalities, Multiple↗
PubMed2002

Intraoperative radiofrequency ablation of the atrium: effectiveness for treatment of supraventricular tachycardia in congenital heart surgery.

BACKGROUND: Supraventricular tachycardia (SVT) is common in surgical patients with congenital heart disease. Ablation and maze operations have been shown to be effective in treating SVT, but these procedures can be complex and time-consuming because of variable anatomy and a thickened right atrium. To simplify and shorten these procedures, we used a long, flexible radiofrequency probe capable of producing long ablation lines quickly and effectively. We report the initial results with this procedure. METHODS: Six patients aged 6 weeks to 40 years with refractory SVT were referred for reoperation for repair of complex congenital heart disease (transposition of the great vessels, Ebstein's anomaly, single ventricle, tetralogy of fallot). Intraoperative radiofrequency ablation was performed in the right atrium for refractory SVT as an adjunct to surgical reconstruction (redo Fontan, right atrial reduction plasty, right ventricular outflow tract reconstruction, tricuspid repair). Lesions were made with a radiofrequency probe using temperatures of 70 degrees C for 60 seconds. Lesions were placed between the coronary sinus and the tricuspid valve, between the tricuspid valve and the inferior vena cava, between the atrial septal defect and the superior and inferior vena cava in patients with intraatrial reentry tachycardia/atrial flutter, and at the location of the accessory pathway in a patient with Wolff-Parkinson-White syndrome. The long, flexible probe has multiple independently controlled segments allowing ablation lesions that conform to the atrial morphology. RESULTS: An average of five intraoperative radiofrequency ablation lesions per patient were made. Average time for ablation was 14 minutes. With up to 25 months' follow-up, 5 patients are in sinus rhythm, and 1 is in a paced atrial rhythm. The patient with Wolff-Parkinson-White syndrome showed no preexcitation after operation. No complications resulting from intraoperative radiofrequency ablation were encountered. CONCLUSIONS: Intraoperative radiofrequency ablation in the atrium is a safe, effective, and expeditious procedure for control of SVT in patients undergoing reoperation for congenital heart disease with refractory SVT.

Adolescent↗
PubMed1986

Coronary arteries in complete transposition: the significance of the sinus node artery.

Hearts from 29 children with complete transposition were examined postmortem to analyze the pattern of the coronary arteries and the origin of the sinus node artery. Four patterns of origin and distribution of the coronary arteries were found. In all, the coronary arteries arose from one or both of the facing sinuses, which were termed sinus 1 and sinus 2. The patterns were left coronary artery from sinus 1 and right coronary artery from sinus 2; anterior descending artery from sinus 1 and right coronary artery and circumflex artery from sinus 2; left coronary and right coronary from sinus 2; and circumflex artery from sinus 2 and right coronary artery from sinus 1. This study highlights the patterns of the coronary arteries in complete transposition and categorizes the variability using a simple classification. It also emphasizes the surgical significance of variations in distribution of the sinus node artery.

Autopsy↗
PubMed1980

Open-heart surgery in the first week of life.

The high mortality of open-heart operations in infants with congenital heart disease has traditionally led to deferral of definitive operation and to use of medical therapy or palliative procedures. The technique of deep hypothermia with circulatory arrest and advances in intensive care have made early repair possible. Since 1973, we have repaired life-threatening but correctable lesions in 11 infants in the first week of life. There are 7 survivors. Four patients had total anomalous pulmonary venous drainage, 2 had truncus arteriosus (1 with aortic arch interruption), and 1 each had pulmonary atresia and intact ventricular septum, critical pulmonary stenosis and intact ventricular septum, D-transposition of the great vessels, tetralogy of Fallot, and left ventricular-aortic tunnel. Postoperative hypoglycemia (less than 50 mg per 100 ml) developed in 4 patients and hypocalcemia (less than 7 mg per 100 ml), in 5. These problems responded appropriately to replacement therapy. Three of the survivors experienced renal failure, a complication subsequently prevented by use of mild hemodilution perfusion. Two patients had major bleeding, which now is prevented by finer suturing and administration of vitamin K and platelets. One infant, moribund with pH of 6.8 when brought to operation, sustained a cardiac arrest with subsequent brain damage. We conclude that newborns with life-threatening malformations can undergo successful repair. Attention must be paid to their unique metabolic demands, and surgical technique must be meticulous. Early operation before clinical deterioration is essential.

Follow-Up Studies↗
PubMed1986

Repair of interrupted aortic arch and associated malformations in infancy: indications for complete or partial repair.

There is uncertainty regarding the best method of repair of interrupted aortic arch. The question is whether to perform primary definitive repair of this anomaly plus the associated defects versus arch repair only and palliation of the intracardiac defects, usually by pulmonary artery banding. Since 1976, 16 infants with interrupted aortic arch have been treated surgically. They were seen at 5.2 +/- 3 days of age and weighed 3.2 +/- 0.7 kg. The interruption occurred between the left carotid and left subclavian arteries (type B) in 9 and between the left subclavian artery and the descending aorta in 7 (type A). Isolated ventricular septal defect (VSD) was the only associated anomaly in 7 and aortopulmonary window, in 4. Two patients had truncus arteriosus type 1. Three had transposition of the great arteries: 1 with VSD and 2 with single ventricle. Prior to 1980, our policy was to palliate all patients. Between 1976 and 1980, 4 infants underwent left thoracotomy with arch repair plus pulmonary artery banding (3, VSD; 7, transposition of the great vessels and single ventricle) with only 1 (25%) survivor. Because of this high mortality, 8 patients with interrupted aortic arch and VSD or aortopulmonary window, seen since 1980, received complete repair with median sternotomy, end-to-end arch anastomosis, and closure of the VSD or aortopulmonary window utilizing profound hypothermia and circulatory arrest. All 8 survived.(ABSTRACT TRUNCATED AT 250 WORDS)

Aorta, Thoracic↗
PubMed1989

Pulmonary artery banding: analysis of a 25-year experience.

A 25-year experience (May 1962 through April 1987) with pulmonary artery banding in 183 patients was reviewed and analyzed. Pulmonary artery banding was performed in a heterogeneous group of patients aged two days to 60 months (median, 10 weeks; mean, 21.8 weeks) and weighing 1.4 to 13.8 kg (mean, 4.2 kg). Diagnosis was ventricular septal defect in 76 (41.5%) and atrioventricular communis in 41 (22.4%). Pulmonary artery banding was also used in patients with d-transposition of the great vessels with ventricular septal defect, double-outlet right ventricle, univentricular heart, tricuspid atresia, and truncus arteriosus. Early death occurred in 39 of 175 patients who underwent pulmonary artery banding at Ochsner Foundation Hospital (22.3%). Definitive operation has been performed in 37 of the patients who underwent pulmonary artery banding since 1979 with excellent outcome in 32 (86.5%). Pulmonary artery banding is a useful palliative procedure for a diverse group of patients with congenital cardiac anomalies and unrestricted pulmonary blood flow. With improved results of primary repair of intracardiac anomalies in small infants, however, pulmonary artery banding should be reserved for severely ill patients with complex lesions not amenable to early definitive correction. Currently, pulmonary artery banding is indicated in patients with excessive pulmonary blood flow and single ventricle or tricuspid atresia. Pulmonary artery banding is also appropriate in certain patients with atrioventricular communis and in patients with muscular or multiple ventricular septal defects. Pulmonary artery banding is an option in patients with ventricular septal defect and coarctation of the aorta.

Constriction↗
PubMed1999

Laparoscopy versus laparotomy in the management of adnexal masses during pregnancy.

OBJECTIVE: To compare the outcome of pregnancy after operative laparoscopy or laparotomy for the management of adnexal pathology during pregnancy. DESIGN: Retrospective comparative study. SETTING: University tertiary care referral center for endoscopic surgery. PATIENT(S): Eighty-eight pregnant women who underwent 93 operations for suspected adnexal pathology at our institute. Laparoscopy was performed during the first trimester in 39 patients. The remaining 54 patients underwent laparotomy, 25 during the first trimester and 29 during the second trimester. INTERVENTION(S): Laparoscopy or laparotomy for the management of adnexal masses during pregnancy. MAIN OUTCOME MEASURE(S): Operative and postoperative maternal complications, miscarriage, congenital malformations, and newborn long-term outcome. RESULT(S): No operative or postoperative maternal complications occurred in the pregnant women who underwent laparoscopic surgery. In this group of 39 women, 5 women had a first-trimester miscarriage and 2 newborns had congenital malformations (hypospadias and cleft lip and palate). Two miscarriages occurred in the first-trimester laparotomy group, and 1 congenital malformation (transposition of the great vessels) was diagnosed in the second-trimester laparotomy group. CONCLUSION(S): Laparoscopic gynecologic surgery appears to be safe during pregnancy, although prospective controlled studies and national registries encompassing larger numbers of cases are needed.

Abortion, Spontaneous↗
PubMed1979

An evaluation of the modified endorectal procedure for Hirschsprung's disease.

During the past 4 yr, 60 patients with Hirschsprung's disease have been treated at our institution. Twenty-eight patients within this group have undergone a modified endorectal pull-through procedure. There are 18 males and 10 females in the series. There are 5 children with total colonic involvement, 3 patients with aganglionosis to the mid-transverse colon, and one child with Hirschsprung's disease extending to the distal jejunum. The associated anomalies have consisted of four cases of Down's syndrome, one of whom also has transposition of the great vessels. There has been no operative-related mortality or late mortality in our series. Two major complications (anastomotic leak following a colostomy closure and incorrect pathological diagnosis of aganglionic level) and five minor complications have occurred. The average follow-up period is 31.3 mo. The functional results postoperatively have been excellent. The 18 patients over the age of 3 yr are completely toilet-trained. The remaining patients are too young to be toilet-trained, but are continent in the sense that they do not soil between bowel movements. Because of the satisfying functional results in this series, we have now expanded our indications for this procedure to patients requiring total colectomy for ulcerative colitis and familial polyposis. During the past 2 yr, 8 patients with ulcerative colitis have undergone the endorectal pull-through together with a total colectomy.

Anal Canal↗
PubMed1999

Radiographic evaluation of the neonate with congenital heart disease.

Remarkable advances in pediatric cardiology have been spurred by the explosion of technologies both in interventional and surgical techniques and the ability to manipulate the genome of experimental animals. After a brief discussion concerning the striking advances in the molecular understanding of congenital heart disease, this article focuses on clues to the diagnosis of congenital heart disease and on chest radiography and common, specific lesions of the neonate such as hypoplastic left heart, transposition of the great vessels, and severe tetralogy of Fallot. The impact of treatment protocols involving interventional cardiology in the neonate also are discussed.

Heart Defects, Congenital↗
PubMed1985

Acute hemodynamic effects of increasing hemoglobin concentration in children with a right to left ventricular shunt and relative anemia.

The short-term effects of increasing hemoglobin concentration were evaluated at cardiac catheterization in seven children (aged 0.3 to 7.5 years) with a right to left ventricular shunt and relative anemia. Diagnoses were tetralogy of Fallot in six and L-transposition of the great vessels with ventricular septal defect and pulmonary stenosis in one. Before and 20 minutes after isovolumic partial exchange transfusion with 20 ml/kg packed red cells, the following variables were measured: hemoglobin, partial pressure of oxygen (PO2), oxygen consumption, oxygen saturation and pressure in the aorta, superior vena cava and right and left atria. After partial exchange transfusion, hemoglobin increased from 13.7 +/- 0.5 to 16.4 +/- 0.4 g/100 ml (p less than 0.001, mean +/- SEM). Aortic PO2 increased from 55.0 +/- 3.5 to 62.0 +/- 4.1 mm Hg (p less than 0.01) and aortic oxygen saturation increased from 84.3 +/- 2.3 to 90.9 +/- 1.3% (p less than 0.002). Effective pulmonary blood flow increased by 17% from 2.72 +/- 0.10 to 3.17 +/- 0.10 liters/min per m2 (p less than 0.01), and right to left shunt decreased by 59% from 1.44 +/- 0.29 to 0.59 +/- 0.10 liters/min per m2 (p less than 0.01). Systemic oxygen transport increased from 658 +/- 48 to 738 +/- 46 ml/min per m2 (p less than 0.002). After partial exchange transfusion, systemic vascular resistance increased from 15.9 +/- 1.1 to 20.0 +/- 1.4 units (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Anemia↗
PubMedDate not supplied

Fetal echocardiography: a large clinical experience and follow-up.

We reviewed our experience with 382 fetal echocardiograms. Complete studies were not possible for three pregnancies because of either fetal position or maternal obesity. Studies were performed for fetal arrhythmia (28%), maternal factors (21%), fetal anomaly (11%) and family history of congenital heart disease (40%). There was a recurrence of heart disease in two of 153 patients (1.3%). Arrhythmia was the most common finding (82 of 382 patients). Premature atrial and ventricular contractions were the most common arrhythmia, and structural defects were present in four of 58 patients (6.8%) with premature contractions. Fetal heart defects (n = 44) were identified in 40 of 382 (10%) referrals. The defects were complete atrioventricular block (13), ventricular septal defect (4), atrioventricular canal (5), cardiac mass (3), ectopia cordis (2), thoracopagus (2), hypoplastic left ventricle (2), hypoplastic right ventricle (2), atrial flutter (2), pulmonic stenosis (1), single ventricle (2), Uhl's anomaly (1), Ebstein's anomaly (1), mitral atresia (1), d-transposition of the great vessels (1), tetralogy of Fallot with absent pulmonary valve (1), and atrial septal defect and ventricular septal defect (1). There were three false positive (99% specificity) and two false negative (95% sensitivity) fetal echocardiograms. The survival rates for referred patients with heart defects was: live born and perinatal survivor, 54%; perinatal death, 31%; still birth, 11%; and termination of pregnancy, 4%. Fetal echocardiography is accurate, and the abnormalities detected appear to be more severe than those detected on newborn screening.

Arrhythmias, Cardiac↗
PubMed1994

Small hyperechoic ventricle: a benign foci of the fetal cardiac sonographic finding?

The objective of our study was to determine the significance of antenatally detected, small hyperechoic foci in the fetal cardiac ventricle. In a 13-month period, we identified 25 fetuses with such a finding, an incidence rate of 0.46%. The hyperechoic foci in 24/25 fetuses were seen during follow-up serial scans. The size ranged from 1 to 6 mm. All had a normal fetal echocardiogram at 20-22 weeks' gestation. Eleven of the 24 neonates had a normal postnatal echocardiogram in the first 4 weeks of birth. One neonate had a previously undiagnosed transposition of the great vessels and no residual hyperechoic focus. Twelve had an echogenic focus located at the level of the chordae tendineae and papillary muscles in the left ventricle. In each of these 12 neonates, there was normal ventricular function determined by measuring the shortening fraction, and a competent atrioventricular valve. When hyperechoic foci are identified, baseline antenatal echocardiography should be performed to confirm their benignity. With an otherwise normal fetal and clinical neonatal evaluation, serial antenatal and confirmatory postnatal echocardiography are unnecessary. A possible relationship to other cardiac anomalies or aneuploidies requires further study.

Journal Article↗