Rapid two-stage repair of S,L,L, ventricular septal defect, pulmonary atresia, Ebstein anomaly of the tricuspid valve, and stenotic pulmonary arteries.

The repair of a corrected transposition of the great arteries, ventricular septal defect, and pulmonary atresia is presented. An Ebstein anomaly of the tricuspid valve, dextrocardia, and severe distortion of the pulmonary arteries complicated the surgical procedure, which was performed in two stages. Reconstruction of the pulmonary arteries and a bidirectional cavopulmonary anastomosis were performed first; Rastelli and hemi-Mustard procedures completed the correction. The rationale and the possible indications of this "one and a half ventricle" repair are discussed.

Surgical retrieval of an embolized central venous catheter in a premature baby.

Embolization of central venous catheter fragments is usually treated with percutaneous interventional techniques, which are difficult to apply in infants with very low birth weight. We surgically removed a catheter fragment in a preterm neonate, to avoid the impending thrombosis of the right pulmonary artery. The operation was performed with a nerve hook introduced through a tiny incision in the vessel's wall. The procedure was well tolerated, and no stricture remains at the site of incision.

Arteriovenous fistulas of the placenta in a singleton fetus with large atrial septal defect and anomalous connection of the umbilical veins.

Arteriovenous fistulas of the placenta rarely occur in singleton pregnancies. This report describes the fetal and neonatal hemodynamic pattern in a singleton gestation in which multiple placental artery-to-vein anastomoses were associated with a large atrial septal defect and a single umbilical artery with an anomalous connection of the persistent right and left umbilical veins. Possible links between the extracardiac vascular malformation and the congenital heart defect are discussed.

Berry syndrome, a complex aortopulmonary malformation: one-stage repair in a neonate.

Successful one-stage repair of a Berry syndrome (interrupted aortic arch, distal aortopulmonary septal defect, right pulmonary artery branch originating from the ascending aorta, and intact ventricular septum) in the neonatal period has been reported in only 2 cases. We report the case of a newborn operated on with deep hypothermic arrest and isolated myocardial perfusion in whom the interrupted aortic arch was corrected by direct anastomosis between the ascending and descending aorta and the aortopulmonary septal defect was treated with reconstruction of the pulmonary trunk and right pulmonary artery, using a flap of aortic tissue. A native pericardial patch was used to reconstruct the ascending aorta.

Echocardiographic assessment of aortic valve replacement with stentless porcine xenografts.

Stentless porcine xenografts (SPXs) implanted in the aortic position have potential hemodynamic advantages over traditional valve prostheses because of the lack of a rigid stent. Twenty-four patients (mean age 59 years) who underwent aortic valve replacement with SPXs were studied by echocardiography early after and 26 +/- 10 months (range 8 to 40) after operation. Peak and mean gradients, as well as aortic valve area, did not change significantly from baseline (16.3 +/- 8 and 9.8 +/- 5.6 mm Hg, and 1.78 +/- 0.63 cm2, respectively) to follow-up study (12.5 +/- 5 and 7.7 +/- 3 mm Hg, and 1.8 +/- 0.65 cm2, respectively). At baseline, color flow Doppler imaging showed aortic valve regurgitation where the leaflets coapted centrally in 17 of 24 patients (trivial, n = 14; mild, n = 3). Besides the central leak, paravalvular regurgitation was seen in 4 patients (trivial, n = 3; mild, n = 1). At follow-up, 18 of 24 patients had aortic valve regurgitation (trivial, n = 11; mild, n = 6; and moderate, n = 1). New valvular regurgitation (graded as trivial, n = 2; mild, n = 2; and moderate, n = 1) was detected in 5 patients, and new paravalvular regurgitation (graded as mild) developed in 1 patient. Two patients underwent repeat operation for valve-related complications: (1) rupture of a valve cusp with acute pulmonary edema, and (2) fibrotic stenosis of the left coronary ostium with unstable angina. In conclusion, this study demonstrates good hemodynamic performance of the SPX in the aortic position.(ABSTRACT TRUNCATED AT 250 WORDS)

[A transesophageal Doppler echo-color study of pulmonary venous flow before and after the correction of valvular defects]. / Studio mediante eco-color Doppler transesofageo del flusso venoso polmonare prima e dopo correzione di vizi valvolari.

AIM OF THE STUDY: To verify changes of pulmonary venous flow pattern before and after surgical or percutaneous correction of valvular heart disease. METHODS: The pulmonary venous flow pattern was studied by transesophageal echocardiography in 27 patients affected with heart valve disease (11 mitral insufficiency, 10 mitral stenosis, 2 aortic stenosis and 4 pulmonary stenosis), before and after surgical or percutaneous correction. Pulmonary venous flow velocity variables measured included peak systolic and diastolic flow velocities (VmaxS and VmaxD), systolic and diastolic velocity time integrals (IS and ID) and their respective ratios (VmaxS/VmaxD and IS/ID). Paired Student's t-test was used for analysis of data; a p value < 0.05 was considered statistically significant. RESULTS: In mitral stenosis and insufficiency, as well as in pulmonary stenosis, the VmaxS/VmaxD and IS/ID ratios were constantly < 1. Aortic stenosis, on the contrary, showed a normal preoperative pattern of pulmonary venous flow, which did not change after correction. All other successful corrections (17 surgeries, 4 angioplasties) were characterised by an increase of VmaxS/VmaxD and IS/ID ratios. (Mitral stenosis: VmaxS/VmaxD 0.80 +/- 0.31 vs 1.4 +/- 0.5, p = 0.006; IS/ID 0.86 +/- 0.77 vs 1.62 +/- 0.62, p = 0.016. Severe mitral insufficiency: VmaxS/VmaxD -0.71 +/- 0.32 vs 1.19 +/- 0.32, p < 0.0001; IS/ID 0.41 +/- 0.19 vs 1.04 +/- 0.31, p = 0.006. Moderate mitral insufficiency: VmaxS/Vmax D 0.38 +/- 0.04 vs 0.95 +/- 0.06, p = 0.001; IS/ID 0.32 +/- 0.05 vs 0.95 +/- 0.07, p = 0.02. Pulmonary stenosis: VmaxS/VmaxD 0.43 +/- 0.23 vs 1.09 +/- 0.35, n.s. e IS/ID 0.49 +/- 0.34 vs 0.92 +/- 0.65, n.s.). Failure to return to a normal pulmonary venous pattern was observed in the 2 cases of partially successful mitral valvuloplasty (one of which was subsequently transformed into a mitral valve replacement with immediate normalisation of the pattern) and in the 2 cases of incomplete relief of a pulmonary stenosis after pulmonary valvuloplasty. CONCLUSIONS: Though preliminary, these observations suggest a high sensitivity of this method and, therefore, a possible role of pulmonary venous pattern studies in the assessment of the efficacy of treatment in mitral and pulmonary valve disease.

Regurgitant flow of mitral valve prostheses: an intraoperative transesophageal echocardiographic study.

To assess the regurgitant characteristics of mitral biologic and mechanical prostheses immediately after implantation, intraoperative transesophageal echocardiography was performed in 27 patients, aged 32 to 69 years, undergoing open-heart surgery for rheumatic heart disease (n = 19), mitral valve prolapse (n = 3), malfunctioning prostheses (n = 3), or periprosthetic leaks (n = 2). The prostheses included 13 biologic (Carpentier-Edwards) and 14 mechanical valves (five Starr-Edwards, five Medtronic-Hall, and four Bjork-Shiley). Physiologic transvalvular regurgitant flow was detected in both biologic and mechanical prostheses. The spatial extent of the regurgitant jets was usually greater in the mechanical than in the biologic valves, and systolic jets, characteristic of each type of valve, were visualized consistently. Trivial periprosthetic jets (PPJs) were observed in many implanted valves (14/27). The median maximal jet area was 0.46 cm2 (range 0.1 to 1.5 cm2). Cardiopulmonary bypass was reinstituted in two patients. In one patient a PPJ was judged extensive enough (area 3.6 cm2) to warrant surgical revision of the implant, but no dehiscence was found. In the other patient a turbulent PPJ (area 5.5 cm2) was associated with a 0.5 cm dehiscence at the surgical inspection. In conclusion, (1) all mitral prostheses exhibit physiologic transvalvular regurgitation, (2) trivial mitral PPJ is a common finding in newly implanted mitral valves and does not require the revision of the implant, and (3) further experience based on larger series of patients is required to determine the maximal acceptable size of a mitral PPJ detected by intraoperative transesophageal echocardiography.

Intraoperative echocardiography by a new miniaturized epicardial probe: preliminary findings.

Miniaturized probes constitute recent progress in the field of epicardial echocardiography. We recently used a new miniaturized probe, derived from a standard transesophageal probe, in a series of 12 adult patients who underwent cardiac surgery in order to test the possibility of obtaining new views for epicardial imaging. This study demonstrates the feasibility and safety of performing intraoperative echocardiography when using a miniaturized epicardial probe. This probe may be placed on a broader epicardial and vascular area, thus overcoming the size limitations of the commonly used epicardial probes. The major limitation found with the miniaturized probe, however, was the inability to obtain a true four-chamber view from the ventricular apex, due to the difficulty of holding the probe motionless between the apex and the diaphragm while the heart is beating. Although extensive experience with larger groups of patients and different pathologies will be required to define the full potential of this new probe, the advent of the miniaturized probe may further expand the applicability of epicardial echocardiography in pediatric patients during surgery for congenital heart disease.

[Echocardiography-guided endomyocardial biopsy]. / La biopsia endomiocardica ecoguidata.

We have performed 20 echocardiography-guided endomyocardial biopsies in 16 patients, totalling 98 bioptic samples. In each case fluoroscopy was available to supplement the echocardiographic findings. The right ventricle was biopsied in 17 cases, the left ventricle in 3. All 3 left ventricular biopsies and 14 out of 17 right ventricular biopsies were satisfactorily guided by echocardiography. An adequate echocardiographic window was not obtained in 3 cases of right ventricular biopsies and the procedures were carried out under fluoroscopy. In 5 cases (25%), totalling 10 samples, echocardiography showed a somewhat different position of the bioptome from that suggested by fluoroscopy, thus guiding a significant repositioning of it. Finally, in one patient, echocardiography promptly visualized a severe pericardial effusion, due to cardiac perforation, thus allowing its immediate drainage.

Long-term results after atrial correction of complete transposition of the great arteries.

This study presents the late results for the first 104 consecutive patients surviving and atrial repair for transposition of the great arteries (TGA) between January 1971 and December 1978 (group 1). Mean follow-up was 12 years (range, 0.1 to 17.7 years). The actuarial survival rate at 18 years was 84.2% (70% confidence limits, 79% to 88%) for simple TGA and 93.7% (70% confidence limits, 84% to 97%) for complex TGA. Nine of the 11 deaths were sudden. Two (2.6%) of the 78 late survivors operated on for simple TGA are in New York Heart Association functional class III or IV versus 4 (26.7%) of the 15 survivors with complex TGA; the other patients are doing very well. To better assess long-term results, we report the findings for randomly obtained electrocardiograms, Holter monitor recordings, radionuclide angiographic studies, and cardiac catheterizations performed in 1987 in a larger group of 159 long-term survivors of atrial repair operated on at Ospedale Riuniti di Bergamo from January 1971 to December 1984 (group 2), which includes all of group 1. The findings confirm that the arterial switch repair is the procedure of choice for complex TGA and that there is a major incidence (approximately 10%) of systemic right ventricular dysfunction and rhythm disturbances after the atrial repair. On the other hand, our late survival rate at 18 years of 84% for simple TGA with 97.5% of the patients in functional class I is a result that should be kept in mind, especially in institutions where the arterial switch is a relatively new approach and presumably is a higher risk to cause early death.

Late left atrioventricular valve insufficiency after repair of partial atrioventricular septal defects: anatomical and surgical determinants.

Risk factors for late left atrioventricular (AV) valve insufficiency, which occurred in 16 (18%) of 90 patients evaluated after repair of partial AV septal defect, were examined. The operative findings in 9 patients undergoing reoperation were also examined. Preoperative left AV valve insufficiency was significantly more common in the group with late left AV valve incompetence, as were associated valvular malformations as a whole and fenestrations of valve leaflets in particular. Conversely, the higher incidence of malformed or malpositioned papillary muscles, accessory clefts, and double-orifice left AV valves in the group with late left AV valve insufficiency did not reach significance. The method of surgical treatment of the septal commissure was not a significant factor. In the group having reoperation, additional valvular malformations were found in association with inappropriate treatment of the septal commissure in 7 patients. The 2 remaining patients had either a directly sutured ostium primum or dilatation of the annulus. Three re-repairs were successful. Five patients required prosthetic valve replacement. Preoperative left AV valve insufficiency and associated valvular malformations are major determinants of late left AV valve insufficiency in partial AV septal defect.

A current appraisal of the Waterston shunt.

Recent reports have suggested that the classical Blalock-Taussig shunt and its prosthetic modification have a low patency rate in neonates. This prompted a review of 58 neonates undergoing a Waterston shunt for cyanotic congenital heart disease. The 4 mm anastomosis was constructed using a calibrated clamp. There were 20 (35%) hospital deaths, of which 3 were directly related to shunt failure. Operative mortality was associated with increasing complexity of the cardiac lesion, emergency operation, weight less than 3 kg and age less than 48 hours. There were 5 late deaths resulting in an actuarial survival of 52% at ten years. There was one case of late occlusion corresponding to a patency of 92% at 2 years. Anastomotic kinking with preferential flow to one lung occurred in 2 (7%) of 28 patients undergoing repeat angiography at 3 and 5 years respectively. Nineteen patients underwent corrective surgery a mean of 27 months after shunt construction. Six (32%) required an angioplastic repair of the pulmonary artery. Six (16%) of the 38 operative survivors required diuretic therapy for excessive pulmonary flow. The high patency ensures that the Waterston shunt continues to play an important role in the management of low weight neonates who require an emergency systemic-pulmonary shunt. The use of a calibrated clamp reduces the incidence of excessive pulmonary flow. Early corrective surgery and shunt dismantling may reduce the necessity for angioplastic repair.