Air travel and adults with cyanotic congenital heart disease.

BACKGROUND: Concern has been expressed that a reduction of partial oxygen pressure during flight in commercial aircraft may induce dangerous hypoxemia in patients with cyanotic congenital heart disease. METHODS AND RESULTS: To evaluate the validity of this concern, the transcutaneous SaO2 was measured in 12 adults with this type of heart disease and 27 control subjects during simulated commercial flights of 1.5 and 7 hours in a hypobaric chamber. Ten of those patients and 6 control subjects also were evaluated during two actual flights of approximately 2.5 hours in a DC-10 and an A-310, respectively. During the prolonged simulated and actual flights, the capillary blood pH, gases, and lactic acid were analyzed in the patients and during one of the actual flights also in the control subjects. During the simulated flights the SaO2 was at all times lower in the patients than in the control subjects. However, the maximal mean actual percentage decrease, as compared with sea level values, did not exceed 8.8% in either patients or control subjects. During the actual flights, this maximal decrease in the patients was 6%. In-flight reduction of the capillary PO2 was considerable in the control subjects but not in the patients. It is our hypothesis that the lack of a significant decrease of the PO2 in the patients might possibly be due to a high concentration of 2.3 diphosphoglycerate in the red cells. The flights had no influence on the capillary blood pH, PCO2, bicarbonate, or lactic acid levels in either patients or control subjects. CONCLUSIONS: Atmospheric pressure changes during commercial air travel do not appear to be detrimental to patients with cyanotic congenital heart disease.

Survival of a neonate after myocardial infarction complicated by late mitral regurgitation.

A neonate born with a normal heart developed acute myocardial infarction at 12 days of age. Trivial mitral regurgitation secondary to fibrosis of posteromedial papillary muscle progressed to heart failure at 6 months of age. Mitral valve annuloplasty improved her condition.

Late re-interventions following arterial switch operations in transposition of the great arteries. Incidence and surgical treatment of postoperative pulmonary stenosis.

Seventy-six patients were studied after arterial switch operation (ASO) between May 1977 and February 1992. Pulmonary artery reconstruction was initially performed by: conduit interposition in 5 patients, direct main pulmonary artery anastomosis and button patches in 60 patients, and pantaloon-like patch repair in 11 patients. Pulmonary stenosis developed in 17 patients (22%), requiring a total of 26 late re-interventions. Re-intervention was required in four out of five patients operated with pulmonary artery conduits, 11 out of 60 with a button patch repair and 2 out of 11 following pantaloon-type repair. In this series pulmonary artery stenosis (PS) involving the pulmonary valve occurred in 9/17 patients. Involvement of the pulmonary valve was related to the technique of pulmonary artery reconstruction. In these patients surgery is necessary. Balloon angioplasty can be a valuable tool when the stenosis is more distal. The incidence of PS was not influenced by the type of reconstruction or the use of Lecompte's maneuver.

Pulmonary venous obstruction following correction for total anomalous pulmonary venous drainage: a challenge.

Pulmonary venous obstruction after surgical correction of total anomalous pulmonary venous drainage (TAPVD) is a serious condition. Pulmonary venous obstruction can be the result of a primary developmental error or is due to post-operative anastomotic stricture and is usually manifest within 6 months of surgery. Prompt restudy is indicated and if a stricture is present, urgent surgical relief is indicated. However, the results are often disappointing with a high early mortality and a significant chance of restenosis.

Management of ventricular septal defect with associated aortic incompetence.

Twenty-two patients with a ventricular septal defect and aortic incompetence underwent surgical repair. Mean age was 9.6 years (range, 9 to 15 years). Mean follow-up was 32.4 +/- 15.8 months. The ventricular septal defect was perimembranous in 19 patients and doubly committed or juxtaarterial in 3. Most showed only a small left-to-right shunt. Mean diameter was 11.9 +/- 4.8 mm. Aortic incompetence was mild in 6 patients, moderate in 4, and severe in 12. In 16 patients closure of the ventricular septal defect and aortic valvoplasty were both performed through the aortic root. There were no deaths. All patients retained normal sinus rhythm. No complete heart block was found. In 3 patients secondary aortic valve replacement was required for severe incompetence; in 1 the cause was bacterial endocarditis, in another technical failure, and in a third progressive incompetence over an 8-month period. All patients showed substantial clinical improvement, marked decrease or disappearance of the valvar incompetence, diminution of the left ventricular end-diastolic diameter, and decreased cardiothoracic ratio. We conclude that primary repair is the operation of choice for this combination of lesions. This can avoid or delay considerably valvar replacement. The left-sided approach proves safe and is our preferred technique.

Complete right bundle branch block after surgical closure of perimembranous ventricular septal defect. Relation to type of ventriculotomy.

The ECGs of a 100 consecutive children who had surgical repair of their ventricular septal defects (VSDs) were analyzed for postoperative right bundle branch block (RBBB). Seventy of them had an atriotomy and the other 23 also a ventriculotomy. The ventriculotomy always consisted of a transverse incision a short distance below the pulmonary annulus. Of these children 93 had a perimembranous VSD and the other 7 a pure muscular defect. The ECG results of the 93 children with perimembranous VSDs were statistically analysed. The incidence of postoperative complete RBBB (CRBBB) in the ventriculotomy group was not higher than in the atriotimy group. Infants operated in the first half year of life were more prone to the development of CRBBB than the older children, probably because the VSDs were relatively larger in the younger than the older children. The risk of postoperative CRBBB was less in the children who had direct suture closure of the VSD compared with those who needed a Dacron patch to close the defect. The data in the literature generally indicate a higher incidence of CRBBB after a ventriculotomy than an atriotomy. The absence of this difference and the lower incidence of CRBBB after a ventriculotomy in our series compared with those of several other authors are suggested to be due to the type of ventriculotomy.

Subcostal two-dimensional echocardiographic recognition of a criss-cross heart with discordant ventriculo-arterial connection.

Two-dimensional echocardiography was performed on a patient with criss-cross atrioventricular (AV) relationship and ventriculo-arterial discordance. A systematic approach from the subcostal area revealed the complex cardiac pathology including the criss-cross AV relationship of right and left heart, indicating that this very abnormal relationship can be identified echocardiographically. The echocardiographic findings were confirmed by angiocardiography. Post-mortem investigation showed that much of the complexity, including the criss-cross AV relationship, had been caused by a secondary leftward torsion of the entire ventricular part of the heart.

The ductus arteriosus in the preterm infant: histologic and clinical observations.

In order to elucidate some of the unexplained phenomena in prolonged patency of the ductus arteriosus in preterm infants, the histology of the ductus was studied in 27 cases. Some of the infants had been treated with indomethacin. Four morphologic maturation stages are distinguished. There was no strict relation between gestational age or birth weight and histologic maturation. Therefore, one cannot predict whether a ductus is likely to be mature at the time of birth. In all infants with clinically diagnosed prolonged patency of the ductus beyond the first week, the immature maturation stage or the permanent patent type was observed. In both stages, reopening after initial closure with indomethacin occurred.

Anatomical correction of transposition of great arteries with persistent ductus arteriosus. One year after operation.

The postoperative data are described of a boy who had a 'switch' operation for transposition of the great arteries with persistent ductus arteriosus more than a year before. The child is living a normal active life. Electrocardiography, echocardiography, and angiocardiography show persistent abnormality of structure and function of the right ventricle but no evidence of reduced coronary blood flow, coarctation at the suture lines of the great arteries, or aortic regurgitation. Reference is made to an identical case operated upon 2 1/2 years ago.

[Effects of prostaglandin in heart abnormalities with needed patency of the arterial canal (clinical and histological study)]. / Effets de la prostaglandine dans les malformations cardiaques avec canal artériel obligatoire. Etude clinique et histologique.

Eight infants with cardiac malformations that were dependent on the patency of the ductus, were treated with Prostaglandin E 1 to reverse the closure of the duct. There was clinical improvement in 7 out of the 8 infants, confirmed by improvement in oxygen saturation and/or blood gases. In four cases the opening of the duct was visualised by angiography immediately after the prostaglandin infusion. Side-effects observed were transient pyrexia, flush reactions, apnoeic spells and one case of renal shock. Microscopic examination revealed in the four cases studied conspicuous edema of the media of the dictus. Because of the edema, the ductal wall is friable which indirectly may account for the severe lesions observed in the ductal wall. Prostaglandine E1 has a place in paediatric cardiology but in view of the observations made on the ductal wall it seems advisable to administer the drug only in a low dose and for a short duration.

Histopathology of the ductus arteriosus after prostaglandin E1 administration in ductus dependent cardiac anomalies.

The histology of the ductus arteriosus was studied after prostaglandin E1 (PGE1) administration in 4 infants with ductus dependent cardiac malformations. Pronounced pathological changes were found in each instance. The changes consisted of oedema of the media with separation of medial components by clear spaces, pathological interruptions of the internal elastic lamina, and intimal lacerations, some of which extended into the media. The findings suggest that PGE1 administration has a profound weakening effect on the structure of the wall of the ductus arteriosus, rendering the vessel prone to laceration.

Effect of E1 type prostaglandin on hypoxemia in a cyanotic congenital cardiac malformation.

Prostaglandin type E1 has been administered on 4 different occasions in a newborn with a ductus-dependent complex congenital cyanotic heart disease. Dramatic improvement of the arterial oxygen concentration followed each prostaglandin infusion. Increased pulmonary circulation and widening of the ductus arteriosus were seen on angiographic examination. Transient flushing of the skin and mild pyrexia were the only complications noted. It is advised that prostaglandin type E1 be used in ductus-dependent heart diseases as an emergency therapy when indicated.

The left ventricle in congenital isolated pulmonary valve stenosis. A morphological study.

The wall thickness of the left ventricle was measured in the hearts of 16 children and 2 adults who died of congenital pulmonary valve stenosis. A right-to-left shunt through a patent foramen ovale had existed in 12 cases and was excluded in the other 6. The thickness of the left ventricular wall and the interventricular septum was increased in the majority of hearts, especially in the older patients. There was a good correlation between wall thickness and histological appearance of the left ventricular myocardium; in 2 cases there was also evidence of left ventricular myocardial fibrosis. The coronary arteries appeared normal in all cases. A direct interaction between the ventricles was thought to be a possible mechanism.