Staged surgical management of hypoplastic left heart syndrome: a single institution 12 year experience.

OBJECTIVE: To describe a 12 year experience with staged surgical management of the hypoplastic left heart syndrome (HLHS) and to identify the factors that influenced outcome. METHODS: Between December 1992 and June 2004, 333 patients with HLHS underwent a Norwood procedure (median age 4 days, range 0-217 days). Subsequently 203 patients underwent a bidirectional Glenn procedure (stage II) and 81 patients underwent a modified Fontan procedure (stage III). Follow up was complete (median interval 3.7 years, range 32 days to 11.3 years). RESULTS: Early mortality after the Norwood procedure was 29% (n = 95); this decreased from 46% (first year) to 16% (last year; p < 0.05). Between stages, 49 patients died, 27 before stage II and 22 between stages II and III. There were one early and three late deaths after stage III. Actuarial survival (SEM) was 58% (3%) at one year and 50% (3%) at five and 10 years. On multivariable analysis, five factors influenced early mortality after the Norwood procedure (p < 0.05). Pulmonary blood flow supplied by a right ventricle to pulmonary artery (RV-PA) conduit, arch reconstruction with pulmonary homograft patch, and increased operative weight improved early mortality. Increased periods of cardiopulmonary bypass and deep hypothermic circulatory arrest increased early mortality. Similar factors also influenced actuarial survival after the Norwood procedure. CONCLUSION: This study identified an improvement in outcome after staged surgical management of HLHS, which was primarily attributable to changes in surgical technique. The RV-PA conduit, in particular, was associated with a notable and independent improvement in early and actuarial survival.

Transoesophageal echocardiography in congenital heart disease.

The development of paediatric transoesophageal ultrasound imaging represents an important advance in the diagnosis and management of the patient with congenital heart disease. Although primary diagnostic transoesophageal studies are seldom indicated in infants and unoperated children, they have an important role in the older child, especially where there has been prior cardiac surgery. Diagnostic studies are most appropriate for abnormalities of venous return, the atria, atrioventricular valves and the left ventricular outflow tract. Two other important areas in which transoesophageal imaging is playing an increasing role in the management of the paediatric patient is in monitoring of surgical repair.

Univentricular heart with systemic outflow obstruction: palliation by primary Damus procedure.

In 24 consecutive infants (19 male and 5 female) with complex forms of single-ventricle physiology and systemic outflow obstruction, a modified Damus operation without the use of exogenous material was undertaken in conjunction with creation of an aortopulmonary shunt 3.5 mm in diameter. The median age at operation was 6 days (range, 1 to 170 days) and the median weight, 3.4 kg (range, 2.6 to 4.6 kg). There were nine early deaths. All 15 survivors (median follow-up, 6.5 months) were clinically well without major systemic ventricular dysfunction or atrioventricular or arterial valve regurgitation. Ten of them have undergone a superior vena cava-pulmonary shunt (one death), and 1 has required patch angioplasty of the aortic arch and innominate artery with revision of the aortopulmonary shunt. The 4 other survivors are awaiting a cavopulmonary shunt. Univariate analysis yielded the chronologic rank for an individual procedure (higher risk of death early in the series), presence of aortic arch atresia, and presence or absence of transposition of the great arteries as predictors of death. This aggressive surgical approach provides excellent early palliation, and because the operation prevents abnormal ventricular hypertrophy from pressure or volume overload, systemic ventricular function is optimally conserved for a future Fontan-type procedure.

Transthoracic versus transesophageal echocardiography in the pediatric patient.

The development of pediatric transesophageal ultrasound imaging represents an important advance in the diagnosis and treatment of the child with congenital heart disease. Although primary diagnostic transesophageal studies are seldom indicated in infants and unoperated children, they have an important role in the older child especially where there has been prior cardiac surgery. Diagnostic studies are most appropriate for abnormalities of venous return, the atria, atrioventricular valves, and the left ventricular outflow tract. Two other important areas in which transesophageal imaging is playing an increasing role in the treatment of the pediatric patient is in monitoring interventional catheterization and in the intraoperative and immediate postoperative monitoring of surgical repair.

Surgery for Ebstein's anomaly: the clinical and echocardiographic evaluation of a new technique.

Ten consecutive patients (age range 4 to 44 years, mean 22) underwent surgical repair of Ebstein's anomaly by vertical plication of the right ventricle and reimplantation of the tricuspid valve leaflets. No patient died during or after operation. Intraoperative postbypass echocardiography documented a good result in nine patients but severe tricuspid regurgitation in one patient, who then underwent prosthetic valve replacement during a second period of cardiopulmonary bypass. Two of four patients who had had right ventricular papillary muscle dysfunction in the early postoperative period showed improved papillary muscle function with concomitant reduction of tricuspid regurgitation 6 months later. All patients were evaluated clinically and by echocardiography 2 to 23 months later. All patients showed clinical improvement, seven by one functional class and three by two classes. All were in sinus rhythm. The mean cardiothoracic ratio decreased by 6% (p less than 0.05). On bicycle ergometry performed in six patients, peak oxygen consumption exceeded 20 ml/kg per min in five. Tricuspid regurgitation diminished in eight patients (by three grades in two patients, by two grades in five and by one grade in one patient); it remained unchanged in two. Comparison of preoperative and postoperative pulsed Doppler flow velocities across the pulmonary valve showed an increase in the peak velocity of flow across the valve (mean 83 +/- 14 versus 97 +/- 11 cm/s, p less than 0.005) and a decrease in the time to peak velocity (mean 130 +/- 16 versus 91 +/- 23 ms, p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

The role of transoesophageal echocardiography in adolescents and adults with congenital heart defects.

Between April 1985 and December 1989, outpatient transoesophageal echocardiography was performed in 133 adolescent and adult patients (14% of all outpatient transoesophageal studies) (age range 11-78 years; weight 30-95 kg) to determine the value of this technique both in establishing the primary diagnosis (62 patients) and in the post-surgical follow up (71 patients) of congenital heart disease. The results were correlated with the findings of precordial echocardiography, catheterization and surgical inspection. Clear advantages of transoesophageal imaging over precordial imaging include: (1) direct identification of atrial appendage morphology in all patients; (2) delineation of systemic and pulmonary venous connections; (3) atrial baffle function (eight patients); (4) better evaluation of the Fontan-type circulation (five patients); (5) improved morphologic assessment of the atrioventricular junction and valves (29 patients); (6) definition of subaortic obstruction (18 patients); and (7) definition of ascending aortic morphology in Marfan's syndrome and supravalvar aortic stenosis (13 patients). The problems encountered with transoesophageal imaging include: (1) limited imaging planes; (2) poor visualization of specific intracardiac regions (antero-apical trabecular septum, right ventricular outflow tract); (3) flow masking behind implanted intracardiac prosthetic material.

Comparative value of transthoracic and transesophageal echocardiography in the assessment of congenital abnormalities of the atrioventricular junction.

Information obtained from transthoracic and transesophageal echocardiography (two-dimensional echocardiography with spectral Doppler and color flow imaging) was compared in 17 patients with major congenital abnormalities of the atrioventricular (AV) junction (10 discordant AV connections, 1 criss-cross connection, 5 absent right connections and 1 absent left connection). The findings by either technique were correlated with findings at cardiac catheterization (12 patients) and at surgery (5 patients). In two of six patients with an absent AV connection as defined by transthoracic echocardiography, transesophageal imaging demonstrated an imperforate AV valve. In 11 of 11 patients with a discordant or criss-cross connection, assessment of AV valve and ventricular morphology (by defining the chordal attachments of both AV valves) was possible with transesophageal echocardiography (3 of 11 patients by transthoracic echocardiography); chordal straddling was detected in 1 patient and excluded in 3 others with an associated inlet ventricular septal defect. Anomalous pulmonary venous connection (one patient), atrial septal defect (three patients) and subpulmonary stenosis (five patients) were better assessed by transesophageal imaging, and atrial appendage morphology could be demonstrated in all. The transesophageal technique was less useful in demonstrating the anterior subaortic infundibulum or aortopulmonary shunt (two patients). Although systemic ventricular function could be assessed by either method with use of short-axis M-mode scans, transesophageal pulsed Doppler interrogation of AV valve and pulmonary venous flow patterns provided clues to diastolic dysfunction of the systemic ventricle.

Comparative roles of intraoperative epicardial and early postoperative transthoracic echocardiography in the assessment of surgical repair of congenital heart defects.

In 94 consecutive patients undergoing surgical repair of congenital heart defects the results of intraoperative (after cardiopulmonary bypass) epicardial two-dimensional and Doppler color flow imaging were compared with those of sequential transthoracic echocardiography performed within 24 h of surgery and again before hospital discharge to define the precise role of intraoperative imaging. In 6 of 7 patients with a residual defect requiring immediate surgical revision, intraoperative imaging correctly identified the defect; spectral Doppler imaging underestimated or did not identify a residual outflow tract gradient in 17 patients. Left atrioventricular (AV) valve regurgitation after repair of complete AV septal defect was underestimated in three patients. Although intraoperative documentation of good ventricular function was usually associated with a good outcome, in three patients poor systemic ventricular function after cardiopulmonary bypass was not associated with early mortality. A minor degree of shunting around the patch was a common finding on epicardial and early postoperative imaging and persisted at the time of hospital discharge in 17 of 46 patients who had undergone patch closure of a ventricular septal defect as part of the surgical procedure. Additional trabecular septal defects were missed on color flow imaging after cardiopulmonary bypass in three patients, one of whom required subsequent reoperation. Although intraoperative two-dimensional and color flow imaging permitted the recognition of the majority of residual defects requiring immediate revision, residual outflow obstruction or AV valve regurgitation was usually underestimated.

Comparative values of the precordial and transesophageal approaches in the echocardiographic evaluation of atrial baffle function after an atrial correction procedure.

Previous methods used to assess atrial baffle function after correction of transposition of the great arteries have included precordial echocardiography and cardiac catheterization. To evaluate whether single plane transesophageal echocardiography might provide additional information, its findings were correlated with information derived from both precordial echocardiography and cardiac catheterization in 15 patients (14 Mustard procedures, 1 Senning procedure) aged 4.2 to 33 years (mean 16.3). Precordial ultrasound with combined imaging, color flow mapping and pulsed Doppler ultrasound visualized the supramitral portion of the common systemic venous atrium in every case but could identify only superior limb obstruction in three of six patients, mid-baffle obstruction in zero of two and inferior limb obstruction in zero of two patients. Transesophageal studies with use of the same range of ultrasound methods demonstrated superior limb obstruction (severe in four, mild in two) in six of six patients, mid-baffle obstruction in two of two and inferior limb obstruction in two of two patients. The entire pulmonary venous atrium was equally well interrogated by either ultrasound approach, with both identifying three cases (two mild, one moderate) of mid-pulmonary venous atrium obstruction. However, individual pulmonary vein velocity profiles could only be recorded by transesophageal pulsed Doppler ultrasound. Precordial studies identified baffle leaks (1 large, 2 small) in only three patients, whereas transesophageal studies identified 11 such baffle leaks (1 large, 10 small), which were multiple in two patients. It is concluded that transesophageal echocardiography provides a more detailed and accurate assessment of atrial baffle morphology and function than is provided by either precordial ultrasound or cardiac catheterization.(ABSTRACT TRUNCATED AT 250 WORDS)

Diagnosis of atrial situs by transesophageal echocardiography.

In a prospective investigation, direct visualization of both atrial appendages was attempted during transesophageal echocardiographic studies in 132 patients with congenital heart disease. High quality cross-sectional images delineating the unique morphologic details of both atrial appendages were obtained in every patient. Abnormal cardiac position such as dextrocardia (four patients) or mesocardia (two patients) did not pose any problems for transesophageal assessment of both atrial appendages. Thus, direct diagnosis of atrial situs was possible in every patient. Atrial situs solitus was present in 127 patients studied. Three patients were found to have situs inversus, one had left atrial isomerism and one had right atrial isomerism. No patient with juxtaposed atrial appendages was encountered. All patients had prior subcostal ultrasound scans for assessment of the morphology and relation of the suprarenal abdominal great vessels and the related patterns of hepatic venous drainage. Patients with abnormal atrial situs had correlative high kilovoltage filter beam radiography for assessment of bronchus morphology. The results of situs determination obtained by either method were in agreement. In this series, transesophageal echocardiography allowed the direct and accurate visualization of both atrial appendages and the determination of atrial situs in all patients studied. Transesophageal echocardiography may prove to be the most reliable in vivo technique for determination of atrial situs.

Transesophageal echocardiography in children with congenital heart disease: an initial experience.

Transesophageal echocardiography with a single plane (transverse axis), dedicated pediatric probe was performed prospectively in 25 anesthetized children undergoing routine cardiac catheterization or intracardiac surgery, to assess the potential role of this technique in the initial diagnosis, perioperative management and postoperative follow-up of children with congenital heart disease. The group ranged in age from 1 year to 14.8 years (mean 6.1) and weight from 6.5 to 52 kg (mean 22.4). Studies were successful in all patients and no complications were encountered. The results of the transesophageal studies (combined imaging, color flow mapping and pulsed wave Doppler sampling) were correlated both with the results of prior precordial studies and the information obtained at cardiac catheterization. Transesophageal echocardiography provided a more detailed evaluation of the morphology and function of systemic and pulmonary venous return, the atria, interatrial baffles, atrioventricular valves and the left ventricular outflow tract. Additional information was obtained in 15 patients (60%). Problem areas for single plane transesophageal imaging were the apical interventricular septum, the right ventricular outflow tract and the left pulmonary artery. The intraoperative use of transesophageal echocardiography allowed assessment of the surgical repair and monitoring of ventricular function and volume status while the patient was weaned from cardiopulmonary bypass. Transesophageal echocardiography in pediatric patients is of additional value in three main areas: 1) the precise morphologic diagnosis of congenital heart disease, 2) perioperative monitoring, and 3) postsurgical follow-up.