The role of endoscopy in the treatment of IV type laryngotracheal cleft complications after surgical repair.

Laryngotracheal cleft (LTC) is a rare congenital anomaly. Severe forms of LTC are usually treated surgically using extracorporeal circulation. Despite tremendous improvement of surgical techniques, postoperative complications are still frequent. We report a comprehensive description of an endoscopic approach to diagnosis, surgical repair and endoscopic treatment of complications after correction in a type IV LTC.

Relation between adiponectin and brain natriuretic peptide in healthy pediatric subjects: from birth through childhood.

BACKGROUND AND AIMS: New biomarkers potentially improve clinical management of cardiovascular disease, but there are gaps in understanding their role during childhood. Adiponectin regulates metabolism and exerts anti-inflammatory/anti-atherogenic effects. The aim of the study was to evaluate circulating levels of adiponectin during postnatal growth and its relationship with Brain Natriuretic Peptide (BNP) in healthy children, a marker of cardiac function known to be increased in childhood. METHODS AND RESULTS: Plasma adiponectin and BNP were measured in 131 healthy children divided into: 43 newborns (0-3 days), 29 neonates (4-30 days), 25 infants (1-12 months) and 34 children (1-12 years). A group of 33 healthy adult subjects (25-60 years) was also studied. Plasma adiponectin in the 131 children resulted significantly higher compared to adult subjects (p < 0.0001). The time-course of adiponectin suggests the design of three age-based intervals: the first until 1 month of age (median 29.07 µg/mL, 11.61-47.01 µg/mL 5°-95° percentiles), the second between 1 and 12 months of age (21.66 µg/mL, 8.83-59.81 µg/mL) and the third for age up to 12 years (13.81 µg/mL, 4.10-28.57 µg/mL). Both adiponectin and BNP exhibited the same trend of a progressive decrease during growth, showing a significant relationship (Spearman's rho = 0.403, p < 0.0001). CONCLUSION: Adiponectin plasma levels in a healthy pediatric population vary as a function of age. Three reference intervals for adiponectin in pediatric subjects have been indicated. The relationship between adiponectin and BNP suggests that the age-dependent profile of circulating adiponectin could also be due to BNP.

Plasma C-type natriuretic peptide levels in healthy children.

C-type natriuretic peptide (CNP) is assuming increasing importance in cardiovascular disease, and in adults its plasma levels are related to clinical and functional disease severity. Data are scarce regarding the reference values for CNP in infancy. Aim of this study was to assess the reference intervals for CNP in human healthy newborns and infants. Plasma CNP was measured in 121 healthy children divided into: 41 newborns (age 0-3 days), 24 newborns (4-30 days), 22 infants (1-12 months) and 32 children (1-12 years). A group of 32 healthy adult subjects (age 64 ± 1 years) was also studied. CNP was measured by a specific radioimmunoassay. Between- and within-assay variability resulted ≤ 30 and 20%, respectively and analytical sensitivity 0.77 ± 0.05 pg/tube. Plasma CNP resulted significantly higher in children than in adult subjects (13.6 ± 1.2 pg/ml vs. 7.4 ± 1.0 pg/ml, p=0.030). When the results were analyzed as a function of the age the reference intervals for plasma CNP resulted: 11.6 ± 2.1 pg/ml for newborns (0-3 days), 16.4 ± 3.7 pg/ml for newborns (4-30 days), 15.4 ± 2.7 pg/ml for infants (1-12 months), 13.6 ± 2.3 pg/ml for children (1-12 years) [p=0.01 newborns (4-30 days) vs. adults; p=0.03 infants (1-12 months) vs. adults]. CNP showed the highest concentrations after 12h of life with a peak between 4 and 5 days of life and with a progressive decline afterwards. According to these data at least five different reference intervals for CNP determinations should be used. These observations may be helpful for future clinical application of CNP in human children.

Intracardiac left atrial tuberculoma in an eleven-month-old infant: case report.

BACKGROUND: Cardiac tuberculosis is rare and usually manifests as tuberculous pericarditis. Involvement of other part of the heart is unusual and descriptions in the pediatric literature are confined to few case reports regarding mainly myocardial tuberculosis. CASE PRESENTATION: We describe a case of pulmonary miliary tuberculosis associated with intracardiac left atrial tuberculoma in an immunocompetent eleven-month-old infant successfully treated with surgery and antituberculous therapy. CONCLUSION: Although unusual, involvement of endocardium in disseminated tuberculosis should be kept in mind.

Cardiopulmonary bypass in ewe's fetus: advances and setbacks in our learning curve.

Fetal cardiac surgery represents a surgical challenge and several centers are attempting to establish a suitable methodology in animals. We present our experience with extra-corporeal bypass procedures in preterm and term sheep fetuses. Twenty-two fetuses (103-139 days gestation, mean 115 days gestation) underwent a 1-hour period of right heart-to-pulmonary artery extracorporeal circulation followed by 1 hour of observation. Animals were divided into group 1 and group 2, according to gestational age (above and below 0.85). Three pumps were used: centrifugal without (group 1) reservoir, centrifugal with (group 2) reservoir, and roller with reservoir (group 2). Experiments were completed in 75% of fetuses in group 1 and in 37% of fetuses in group 2. Bleeding was the main cause of failure, especially for group 2. A slow deterioration of blood gas status was noted in group 1, while this trend could be partially reversed in group 2 with corrective measures. Complete heart bypass could not be achieved in either group, and residual fluctuations in arterial pressure were observed. During bypass, body temperature decreased more in group 2 than in group 1. We conclude that cardiac bypass is feasible over a short period in near-term fetuses. A successful outcome may also be obtained in younger fetuses, but better measures need to be implemented for the prevention of surgical bleeding.

Tetralogy of fallot with left heart hypoplasia, total anomalous pulmonary venous return, and right lung hypoplasia: role of magnetic resonance imaging.

We report a rare case of tetralogy of Fallot with total anomalous pulmonary venous return, left heart hypoplasia, right lung hypoplasia, and left ocular-mandibular synchinesia (Marcus-Gunn phenomenon), correctly diagnosed by cardiovascular magnetic resonance imaging and successfully operated by modified Glenn anastomosis.

Mechanical valves in the pulmonary position: a reappraisal.

OBJECTIVES: To evaluate midterm results of mechanical valves in pulmonary position in patients with pulmonary regurgitation and right ventricular dysfunction as an alternative to bioprostheses. PATIENTS: Mechanical valves (six tilting disc valves and two bileaflet valves) were implanted in eight patients previously operated on for tetralogy of Fallot (n = 7) and truncus arteriosus (n = 1), with severe right ventricular dysfunction caused by massive pulmonary regurgitation. RESULTS: All patients survived prosthesis implantation and are currently well. At follow-up (3 months to 9 years), they do not show signs of valve failure, and right ventricular function has dramatically improved in all but one, who still shows moderate ventricular hypokinesia. CONCLUSION: After operative correction of congenital heart defects in selected patients who show severe dysfunction of the right ventricle caused by pulmonary regurgitation/stenosis, mechanical valves may represent an alternative to bioprosthetic valves. The selection of the valve type is still a matter of debate. However, according to literature data, complications seem to have occurred only in patients with bileaflet mechanical valves in the pulmonary position, whereas no thromboembolic episodes or valve failure is reported in subjects with tilting disc valves in the right ventricular outflow. Tilting disc valves might perform better in the right ventricular outflow than bileaflet valves.

Surgical closure of muscular ventricular septal defects using double umbrella devices (intraoperative VSD device closure).

OBJECTIVES: Surgical closure of some muscular ventricular septal defects has been proven to be difficult. In order to simplify the surgical technique we have used intraoperatively Rashkind double umbrella devices to occlude muscular ventricular septal defects. METHODS: On the basis of haemodynamic and echocardiographic study five children aged 4, 6, 7, 14 and 41 months were considered suitable candidates for intraoperative closure of muscular ventricular septal defects (midmuscular in three cases, apical in two) by Rashkind devices. Three of them had previously undergone pulmonary artery banding at 10, 11 and 41 days, respectively. During hypothermic cardiopulmonary by pass a delivery system was introduced across the tricuspid valve into the right ventricle and then passed through the ventricular septal defect; the distal umbrella of a 17 mm device was opened in the left ventricular cavity; a traction was applied to the introducer and the proximal umbrella was opened on the right side straddling the interventricular septum; the device was then secured on the right side by few stitches. In one case because of the wide diameter of the ventricular septal defect two umbrellas were used. The surgical procedure was completed with debanding and/or closure of other defects close to the aortic or tricuspid valve. RESULTS: Immediate results, tested by epicardial or transesofageal echo, showed a minimal residual shunt in 4 patients and a moderate shunt in one. No early deaths occurred. A complete atrioventricular block developed in 1 patient who had an additional perimembranous defect closed with a prosthetic patch: a permanent pace maker was inserted 3 months after the operation. There was a late death for untractable right ventricular failure in 1 patient who had a large residual shunt erroneously considered moderate. In this patient, the size of the defect was underestimated both preoperatively then intraoperatively. The four survivors are doing well with no signs of hemodynamically significant residual shunts. CONCLUSIONS: The use of Rashkind umbrella devices for closing intraoperatively muscular defects can be helpful to standard surgical techniques when technical problems make patch closure difficult. Its use avoid the need of left ventriculotomy. Careful definition of the size of the defect is mandatory to select suitable candidates.

[Enoximone in weaning from mechanical circulation support in pediatric patients]. / L'enoximone nello svezzamento da supporto meccanico di circolo in pazienti pediatrici.

OBJECTIVE: To assess and record the response to continuous infusion of the phosphodiesterase inhibitor enoximone during weaning from mechanical circulatory support (MCS) and to verify the possibility of success with this indication in pediatric patients. DESIGN: Retrospective study. SETTING: Pediatric cardiac surgery intensive care unit. PATIENTS: Two pediatric patients operated for complex congenital heart disease with low cardiac output syndrome in the immediate postoperative period, evolved in cardiocirculatory arrest despite massive inotropic pharmacological support, and then assisted by mechanical circulatory support. INTERVENTIONS: Weaning from mechanical circulatory support with continuous infusion of enoximone, only in one case preceded by a loading dose and associated with catecholamine infusion. MEASUREMENTS AND MAIN RESULTS: During weaning hemodynamic parameters (LAP, CVP, MAP, HR), SvO2, diuresis, rectal and cutaneous temperatures were assessed and recorded. A serial echocardiographic assessment of left ventricular systolic and diastolic diameters and ejection fraction (EF%) has also been performed every 12 hours. Weaning from MCS using enoximone as inotropic support was possible in both cases. CONCLUSIONS: Enoximone proved to be useful in weaning from MCS in two pediatric patients, despite the difficulty to assess its effect in one of the two cases in which enoximone was used together with high dosages of other inotropic drugs. These initial positive results urge us to further investigate applications of this drug in pediatric patients.

Thyroid hormones homeostasis in pediatric patients during and after cardiopulmonary bypass.

The concentrations of thyroid hormones were measured in 14 pediatric patients before, during, and after cardiopulmonary bypass. The ages of the patients ranged between 18 months and 14 years. Patients were kept normothermic, or moderate or deep hypothermia was induced depending on the specific pathologic condition involved. A marked reduction in the levels of total triiodothyronine, total thyroxine, free triiodothyronine, and thyroid-stimulating hormone, and in the ratio of free triiodothyronine to free thyroxine was detected during the time frame of the study. The minimum levels of each hormone were reached between 12 and 48 hours after cardiopulmonary bypass, indicating that changes in thyroid function and in the conversion of thyroxine to triiodothyronine are triggered by cardiopulmonary bypass and represent specific phenomena, and that these changes are progressively exacerbated during the post-operative period. The thyroid-stimulating hormone level was markedly reduced versus its baseline values (24% +/- 0.13%), despite low levels of both total (40% +/- 18%) and free (39% +/- 20%) triiodothyronine: it returned to its preoperative level by the third postoperative day, but both the total (75% +/- 10%) and free (74% +/- 3%) triiodothyronine levels remained below their baseline values for 7 days postoperatively. Neither hemodilution nor hypothermia was responsible for the alteration observed. We conclude that pediatric patients undergoing cardiopulmonary bypass manifest changes in hormone metabolism similar to those seen in adult patients. These changes increase progressively during the postoperative period, and are still present 7 days postoperatively. The exact mechanism responsible for causing these changes is not thoroughly understood. Whether triiodothyronine replacement therapy is beneficial or deleterious remains controversial.

Indirect radiographic and angiographic signs of isolated left superior vena cava in congenital heart disease.

Blood that drains into the right atrium through an isolated left superior vena cava (LSVC) can be a complicating condition during cardiac surgery. In cases of LSVC, a double shadow can be present on standard chest X-ray over the left mediastinum. A notch along the lower contour of left atrium has been previously reported by Owen et al. in 4 cases as an indirect sign of this LSVC anomaly. These two indirect signs were evaluated in 135 (4.5%) cases of LSVC, viewing 89 chest radiographs and 40 angiograms. Their incidence, separately (17-45%) or in combination (9-20%), was observed to be frequent enough to justify a systematic search of these signs in open-heart surgery candidates.

Absent right superior vena cava and persistent left superior vena cava. Clinical and surgical considerations.

From 4,100 patients who had cardiac catheterization for congenital heart disease and " normocardia ", 6 were found to have a persistent left superior vena cava ( PLSVC ) and an absence of right superior vena cava ( RSVC ). Because of the rarity of this finding (1.46%) the clinical and surgical implications are considered. Four of them were successfully operated upon.

The syndrome of congenital absence of the pulmonary valve: pathological, clinical and surgical considerations.

Four cases of congenital absence of the pulmonary valve are described. Three of them underwent successful operation. The fourth case, operated on in the first days of life, died because of severe respiratory distress. After examining the embryological aspects of this malformation, its treatment is discussed in relation to particular respiratory problems in infants and to the possibility of inserting prosthetic valves in children undergoing elective surgical treatment.