E. Harinck

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.

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 Lecomptes maneuver.

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.

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

SummaryTwo-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.

Arrhythmias after surgical correction of complete transposition—follow-up into adolescence

The long-term natural progression of cardiac rhythm and the incidence of serious arrhythmias in relation to previous procedures and associated heart defects were studied in a group of 76 patients after an arterial switch operation for complete transposition and compared to a group of 24 patients who had undergone intraatrial corrections (Mustard or Senning operation). Standard and 24-hour ambulatory electrocardiograms were reviewed. Mean follow-up was 7.9 years (range 2-16) after an arterial switch and 15.2 years (range 6-26) following the Mustard or Senning procedures. One patient died after an arterial switch from pulmonary hypertension (age 9.9 years), and three patients died suddenly, presumably from arrhythmias, following atrial redirection procedures (ages 13, 18 and 20 years). Symptomatic arrhythmias were not seen after the arterial switch operation. Three patients required pacemaker implantation after atrial redirection, and a further two required medication to control tachyarrhythmias. Survival analysis of sequential electrocardiograms showed a mean maintenance of sinus rhythm during 12.9 years (95% confidence interval 11.4-14.5) after the arterial switch and 9.0 years (7.3-10.7) after atrial procedures (p=0.003). Development of heart rate was significantly different (p=0.001), with higher rates in adolescents after an arterial switch. Twenty-four-hour recordings were abnormal in five of 72 patients following arterial correction, disclosing excessive ventricular extrasystoles in four (three monomorphic, one polymorphic) and a wandering pacemaker in one. After atrial procedures, 11 of 19 were abnormal (p

Two-Dimensional Contrast Echocardiography in the Study of Ventricular Septal Defects

Isolated ventricular septal defects (VSDs) can usually be diagnosed under normal circumstances by physical examination, electrocardiogram, phonocardiogram, and chest X-ray. If necessary, confirmation is obtained by cardiac catheterization and angiocardiography. The exact location of the defect may be obtained by angled left ventricular angiocardiography [1]. To achieve angiocardiographic localization several X-ray films are often necessary.

Criss-Cross Heart

The term criss-cross heart denotes an uncommon malformation whereby the pulmonary and systemic bloodflows cross each other at atrioventricular level (1). In the case of atrioventricular concordance the right ventricle is situated superiorly to the left ventricle with a horizontally positioned interventricular septum. Blood from the right atrium empties into the right ventricle in a right-inferior to left-superior direction. The atrioventricular connection on the left side, through which the blood stream passes in a posterior to anterior direction, lies inferior to that of the right side.

Supra pulmonary stenosis after arterial switch operation for transposition of the great arteries

Up until 25 years ago, the mortality rate of untreated transposition of the great arteries was about 90% during the first year of life. The Rashkind balloon atrio-septostomy [5], introduced in 1966, increased effective flow in both circulations and, therefore, diminished cyanosis. This decrease in cyanosis was associated with a significant decrease in the mortality rate. However, the blood exchange at atrial level does not keep up with the increasing flow dimension of children during growth. The Mustard operation, which establishes an atrial switch [4] was developed, and proved to have a low operative mortality rate; it also allows a total separation of pulmonary and systemic circulation. The atrial switch remained, therefore, the operation of choice in the following two decades. Initial enthusiasm for this operation, however, subsided when complications such as obstruction of the venous pathways, arrhythmias, and right-ventricular dysfunction caused a staggering increase in late mortality. The development of the arterial switch operation [1] suggested a new surgical treatment for patients with transposition of the great arteries without the previously stated severe complications.

Ductus Arteriosus and Aorto-Pulmonary Window

The ductus arteriosus is a large channel through which blood flows from the main pulmonary artery to the descending aorta before birth. After birth the wall of the ductus contracts and closure usually occurs in the first few days of life.

Atrial Septum, Atrial Septal Defect and Anomalous Pulmonary Venous Drainage

Isolated atrial septal defect is seldom diagnosed in the early months of life. The compliance of the right ventricle slowly increases above that of the left ventricle. Consequently, the L-R atrial shunt gradually develops. Furthermore, the L-R shunt in atrial septal defect rarely causes clinical symptoms. The patient is commonly referred to the pediatric cardiologist for analysis of a cardiac murmur. An ejection murmur over the pulmonary area with typical fixed splitting of the second sound with a loud pulmonary component is usually the first sign of an atrial septal defect with a significant L-R shunt. In these cases the chest X-ray reveals increased pulmonary vascular markings and cardiomegaly. The cardiomegaly is mainly due to the dilated right ventricle which is readily observed by echocardiography. The absence of evidence of pulmonary hypertension on the pulmonary valve M-mode echocardiogram and the possible presence of paradoxical septal motion indicate that volume overload is the only cause of the right ventricular dilatation. Two-dimensional echocardiography reveals normal attachment of the tricuspid valve without a significant prolapse. The atrial septal defect is best visualized directly in the subcostal four chamber view because in this cross-section the ultrasound beam has a more perpendicular relation with the atrial septum compared with other views. The apical four chamber view (2) and the parasternal short axis view, at the level of the great arteries (3), may also be used but ultrasound coming from these windows tends to pass parallel to the atrial septum. Therefore, central septal ‘dropouts’ easily occur, particularly when the overall resolution is inadequate or the gainsettings are not properly adjusted.