Mechanical circulatory support in pediatric patients.
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Cardiac Output, Low↗
Biomedical subjects
M Beghetti
Publications and source records attributed to M Beghetti.
Aorticoright atrial tunnel.
Two unusual cases of aorticoright atrial tunnel are described. Both patients were referred to our institution for evaluation of a continuous heart murmur best heard along the right upper sternal border. Ascending aortography showed the tunnel taking its origin from the aortic root and entering the right atrium through a tortuous link. Both patients underwent surgical closure. In addition, a review of similar cases in the literature is presented.
Adolescent↗
Anterior mitral leaflet prolapse as a primary cause of pure rheumatic mitral insufficiency.
BACKGROUND: This study was designed to revise the mechanisms and repair techniques of anterior mitral leaflet prolapse observed during the correction of pure rheumatic mitral regurgitation in children. METHODS: From March 1993 to May 1998, 36 children suffering from pure rheumatic mitral regurgitation due to anterior leaflet prolapse underwent mitral valve repair. The mean age was 12.5 years (range, 6 to 16 years). Anterior leaflet prolapse was due to chordal elongation in 25 patients (group A), chordal rupture in 6 patients (group B), and retraction of anterior secondary chordae tendineae, creating a V-shaped deformity in the middle of the anterior leaflet, thus moving the free edge of the anterior leaflet away from the coaptation plane, in 5 patients (group C). Chordal shortening, transposition, and resection of anterior secondary chordae tendineae were used to correct anterior leaflet prolapse according to the predominantly responsible mechanism. RESULTS: All patients were available for clinical follow-up, which ranged from 6 months to 5 years (mean follow-up, 3 years). Echocardiographic studies were obtained until the 3rd postoperative month, and all patients showed significant improvement in their left ventricular and atrial dimensions. There was one late death related to endocarditis. Two patients in group C who had mitral valve repair underwent mitral valve replacement on the 19th and 24th postoperative months, respectively, because of failure of mitral valve repair. CONCLUSIONS: Mitral valve repair for pure mitral regurgitation due to rheumatic anterior leaflet prolapse can be performed safely for all types of mechanisms. Although the techniques we used provide stable short-term results in each of these groups, midterm results are better in groups A and B, where tissue thickening is less important, recurrences of rheumatic carditis are lower, and the interval between the first rheumatic attack and the surgical procedure is shorter than in group C.
Adolescent↗
Etiology and management of pediatric chylothorax.
OBJECTIVES: To determine the incidence and etiology of chylothorax and to assess our therapeutic management approach. STUDY DESIGN: We reviewed 51 patients diagnosed with chylothorax over a 12-year period. Cause, interval between operation and diagnosis, duration of chylothorax, and total volume loss per weight were recorded. RESULTS: Chylothorax was diagnosed in 46 children after cardiothoracic surgery, giving an incidence of 2.5% (46/1842); in 1 child chylothorax occurred after chest trauma, and in 4 the chylothorax was congenital or a manifestation of lymph angiomatosis. Three etiologic groups were identified: group 1, direct injury to the thoracic duct (33/51 = 65%); group 2, thrombosis and/or high venous pressure in the superior vena cava (14/51 = 27%); and group 3, congenital (4/51 = 8%). Conservative treatment was the only treatment in 80% of the patients. Surgical procedures consisted of 4 ligations of the thoracic duct, placement of 7 pleurodesis shunts, and placement of 2 pleuroperitoneal shunts. Patients in groups 2 and 3 were at higher risk for failure of conservative treatment (P <. 005). Longer duration of chylothorax and higher volume of drainage were present in group 2 compared with group 1 (P <.01). CONCLUSION: Conservative treatment was successful in 80% of the patients with our management approach. Prevention, early recognition, and treatment of potential complications, such as superior vena cava thrombosis or obstruction, may further improve success of conservative treatment. Congenital chylothorax seems different and may require a specific approach.
Angiomatosis↗
Comparison of hyperventilation and inhaled nitric oxide for pulmonary hypertension after repair of congenital heart disease.
BACKGROUND: Pulmonary hypertension is associated with congenital heart lesions with increased pulmonary blood flow. Acute increases in pulmonary vascular resistance (PVR) occur in the postoperative period after repair of these defects. These increases in PVR can be ablated by inducing an alkalosis with hyperventilation (HV) or bicarbonate therapy. Studies have shown that these patients also respond to inhaled nitric oxide (iNO), but uncertainty exists over the relative merits and undesirable effects of HV and iNO. HYPOTHESIS: Alkalosis and iNO are equally effective in reducing PVR and pulmonary artery pressure (PAP) in children with pulmonary hypertension after open heart surgery. SETTING: Critical care unit of a tertiary care pediatric hospital. DESIGN: Prospective, randomized, crossover design. PATIENTS: Twelve children with a mean PAP > 25 mm Hg at normal pH after biventricular repair of congenital heart disease. INTERVENTIONS: Patients were assigned to receive iNO or HV (pH > 7.5) in random order, and the effect on hemodynamics was measured. Each treatment was administered for 30 mins with a 30-min washout period between treatments. Finally, both treatments were administered together to look for a possible additive effect. MEASUREMENTS AND MAIN RESULTS: Cardiac output and derived hemodynamic parameters using the dye dilution technique. Hyperventilation, achieved by an increase in ventilator rate without a change in mean airway pressure, decreased Pa(CO2) from a mean (SD) of 43.7+/-5.3 to 32.3+/-5.4 mm Hg and increased pH from 7.40+/-0.04 to 7.50+/-0.03. This significantly altered both pulmonary and systemic hemodynamics with a reduction in PAP, PVR, central venous pressure, and cardiac output and an increase in systemic vascular resistance. In comparison, iNO selectively reduced PAP and PVR only. The reduction in PVR was comparable between treatments, although addition of iNO to HV resulted in a small additional reduction in PVR. An additional decrease in PAP was seen when HV was added to iNO, attributable to a reduction in cardiac output rather than a further decrease in PVR. CONCLUSIONS: Inhaled NO and HV are both effective at lowering PAP and PVR in children with pulmonary hypertension after repair of congenital heart disease. The selective action of iNO on the pulmonary circulation offers advantages over HV because a decrease in cardiac output and an increase in SVR are undesirable in the postoperative period.
Administration, Inhalation↗
Bullous emphysema after Legionella pneumonia in a two-year-old child.
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Anti-Bacterial Agents↗
First intention high-frequency oscillation with early lung volume optimization improves pulmonary outcome in very low birth weight infants with respiratory distress syndrome.
OBJECTIVES: The lack of decline in chronic lung disease of prematurity despite the generalized use of surfactant and alternative modes of ventilation such as high-frequency oscillation (HFO) has been attributed to some misunderstanding of how HFO has to be used. We used a new ventilatory strategy in very low birth weight (VLBW) infants, by initiating HFO immediately after intubation and attempting early lung volume optimization before surfactant was administered. STUDY DESIGN: The outcome of 32 VLBW infants, managed with first intention HFO over a period of 24 months (September 1, 1996 and August 31, 1998) was compared by chart review with 39 historical controls, consecutively managed with conventional mechanical ventilation (CMV) over a period of 24 months (January 1, 1994 and December 31, 1995). SETTING: An 11-bed tertiary care pediatric and neonatal intensive care unit of a university teaching hospital. RESULTS: The 2 groups of patients were similar in demographic distribution of birth weight, gestational age, race, and gender. Patients on first intention HFO were ventilator-dependent (median [95% confidence interval]: 5 [3-6] vs 14 [6-23] days) and oxygen-dependent (12 [4-17] vs 51 [20-60] days) for a shorter time than patients on CMV. The incidence of chronic lung disease at 36 weeks of gestational age was significantly lower in the HFO group compared with the CMV group (0% vs 34%). CONCLUSIONS: First intention HFO with early lung volume optimization shortened the need for respiratory support and improved pulmonary outcome of VLBW infants with respiratory distress syndrome significantly.
Drug Combinations↗
[Follow-up of patients treated by conduits between the right ventricle and the pulmonary artery].
Between 1981 and 1998, 77 right ventricle-pulmonary artery conduits were implanted in 67 patients (37 boys, 30 girls, average age 6.3 years; range: 3 months to 17 years). The diagnoses were transposition of the great arteries with ventricular septal defect and obstruction of the pulmonary outflow tract (N = 22), tetralogy of Fallot (N = 16), truncus arteriosus (N = 9), double outlet right ventricle with pulmonary stenosis or atresia (N = 8) and agenesis of the pulmonary valve with pulmonary stenosis (N = 2). The implanted conduits were homografts in 50 cases (43 aortic and 7 pulmonary), 11 valved Dacron grafts, 4 valved polystans grafts and 2 non-valved conduits. The average follow-up period was 3.6 years (range: 1 month to 17 years). Early death was observed in 8 patients (12%) and late death in 6 patients (9%). The 5, 10 and 15 year survival rates were 78.4%, 65.3%, and 65.3%, respectively. The conduits had to be replaced in 10 patients (15%). The non-replacement rate of all conduits at 5, 10 and 15 years was 81.4%; 40.7% and 40.7%, respectively. The causes of replacement were pure stenosis (54.5%), pure regurgitation (9%) and mixed stenosis and regurgitation (27.2%). These results are comparable to other published series showing a 15 year survival rate of 65% and a 59% reoperation rate at 15 years. Homografts have a slightly longer life compared with valved Dacron conduits but the difference is not statistically significant.
Adolescent↗
Morphologic and functional evaluation of congenital heart disease by magnetic resonance imaging.
Magnetic resonance imaging (MRI) has evolved sufficiently to be recognized as a useful complementary noninvasive method to echocardiography in the evaluation of congenital heart disease (CHD). In some cases, MRI is superior to other imaging modalities, particularly in the evaluation of thoracic aortic anomalies and in defining the anatomy of central pulmonary arteries; it is also the procedure of choice in the postoperative follow-up of patients with CHD. Recent technological advances permit not only morphological evaluation (provided by spin-echo and MR angiographic techniques) but functional and flow information (provided by fast cine-GE and velocity-encoded sequences), causing it to be recognized by pediatric cardiologists and cardiac surgeons as an unavoidable technique for pre- and postoperative evaluation of some CHD. This review describes the various techniques used in the evaluation of CHD with emphasis on recent developments as well as recognized clinical applications. J. Magn. Reson. Imaging 1999;10:639-655.
Adult↗
Inhaled nitric oxide differentiates pulmonary vasospasm from vascular obstruction after surgery for congenital heart disease.
OBJECTIVE: To evaluate whether a trial of inhaled nitric oxide (NO) differentiates reversible pulmonary vasoconstriction from fixed anatomic obstruction to pulmonary blood flow after surgery for congenital heart disease in patients at risk for pulmonary hypertension. DESIGN: Prospective cohort study. SETTING: Tertiary care children's hospital. PATIENTS: 15 neonate and infants with elevated pulmonary artery or right ventricular pressure or with clinical signs suggestive of high pulmonary vascular resistance in the early postoperative period following repair of congenital heart disease. INTERVENTION: 30-min trial of 40 ppm inhaled NO. RESULTS: 5 patients responded to inhaled NO, 2 patients were weaned from extracorporeal support with NO. Four were maintained on continuous inhaled NO for 3 to 5 days. All the responders survived. Ten patients did not respond to NO. An important anatomic obstruction was found with echocardiography or angiography in all 10 patients. Reintervention was performed in 6/10 (4 stent placement, 1 balloon angioplasty of pulmonary arteries and 1 revision of systemic to pulmonary shunt). Six of the nonresponders died. CONCLUSION: A trial of inhaled NO after cardiac surgery in neonates and infants may be useful to differentiate reversible pulmonary vasoconstriction from fixed anatomic obstruction and may provide useful information if temporary support with extracorporeal membrane oxygenation is considered. Failure to respond to inhaled NO should prompt further investigations to rule out a residual obstruction.
Administration, Inhalation↗
Fungal endocarditis in critically ill children.
UNLABELLED: All cases of infective endocarditis occurring from January 1990 to December 1996 at our institution were reviewed, with a special focus on fungal endocarditis. Five critically ill children with fungal endocarditis and eleven children with bacterial endocarditis were recorded. The proportion of fungal endocarditis in our series was 5/16 (31%) and Candida albicans (4/5) was the most common fungal pathogen. Only one patient required heart surgery because of a loose patch but all the others were treated only by medical management for cure. The hospital survival rate was 80% (4/5) and the overall long-term survival rate was 60% (3/5) with only one death directly related to fungal infection. CONCLUSION: Despite the small number of cases, a sole medical approach including amphotericin B and long-term fluconazole prophylaxis for the treatment of fungal endocarditis in critically ill children seems to offer an alternative to surgical treatment which may be kept for failure of medical treatment.
Adolescent↗
Local low-dose urokinase treatment of acquired intracardiac thrombi in preterm infants.
We report three cases of intracardiac thrombi in preterm infants of very low birth weight, in whom local low-dose urokinase treatment achieved complete thrombolysis without any signs of systemic fibrinolytic activity or side-effects. This new treatment strategy seems to be safe, requires minimal monitoring of fibrinolytic activity, and may be a new option for thrombolysis in high-risk patients such as premature infants, patients recently operated on, and patients presenting with other contra-indications for systemic fibrinolytic therapy.
Female↗
Ascending aortic aneurysm associated with aortic insufficiency due to Takayasu's arteritis.
We report the case of a child with an ascending aortic aneurysm associated with aortic insufficiency. Histopathological examination of the ascending aorta and aortic valve showed findings in favor of Takayasu's arteritis, and subsequent evaluation of the entire aorta demonstrated the presence of multiple steno-occlusive lesions. This unusual clinical problem in the young population is discussed with regard to other eventual pathologies that should be taken into account in the differential diagnosis.
Aortic Aneurysm↗
Unilateral total and contralateral partial pulmonary agenesis associated with total anomalous pulmonary venous drainage.
A term newborn presented with total right and partial left lung agenesis associated with total anomalous obstructed left pulmonary venous drainage. This unique association with a dramatic prognosis is thought to be due to a developmental insult early in the embryonic formation of the lung and its supporting vasculature.
Abnormalities, Multiple↗
Aortic valve repair by cusp extension with the use of fresh autologous pericardium in children with rheumatic aortic insufficiency.
OBJECTIVES: Our goal was to evaluate the midterm results of aortic valve repair by a more sophisticated tailoring of cusp extension-taking into account the dimensions of the native aortic cusps-with the use of fresh autologous pericardium. PATIENTS AND METHODS: Forty-one children who had severe rheumatic aortic insufficiency (mean age 11.5 +/- 2.7 years) underwent aortic valve repair by means of this cusp extension technique over a 5-year period. Twenty-four of them underwent concomitant mitral valve repair for associated rheumatic mitral valve disease. All children were then followed up by transthoracic echocardiography before discharge, at 3 and 6 months after the operation, and at yearly intervals thereafter. RESULTS: Follow-up was complete in all patients and ranged from 3 months to 5 years (median 3 years). No operative and no early postoperative deaths occurred. Only 1 patient died, 9 months after the operation, of septicemia and multiple organ failure. Actuarial survival was 97% at 1 year and has remained unchanged at 3 years. On discharge, the degree of aortic insufficiency was grade 0 for 27 children and grade I for 14. Exacerbation of aortic insufficiency from grade I to grade II was observed in only 1 patient, and none of the children required reoperation for aortic insufficiency during the follow-up period. Mean peak systolic aortic valve gradients at discharge were lower than preoperative values (P =.04), and no significant increase in the peak systolic transvalvular gradient was detected thereafter during the follow-up period. Mean left ventricular dimensions were significantly reduced at discharge when compared with preoperative values (P <.0001). CONCLUSIONS: Functional results of aortic valve repair with cusp extension using fresh pericardium have been satisfactory at medium term, particularly in children with a small aortic anulus at the time of initial repair, because the expansion potential of fresh autologous pericardium is equivalent to that of the growing sinotubular junction and aortic anulus diameters.
Adolescent↗
[The pediatrician managing a child with heart disease].
The pediatrician can and must play a pivotal role in the care of children with heart disease. He must provide the preventive care and health supervision as for all children, provide advice to the patient and the family in regards to everyday life, monitor for potential complications or deterioration of the cardiac lesion, and finally play a central role for patients with multiple problems and a chronic illness. The specialist must actively support the generalist with his experience and provide advice for each particular patient. This collaboration should allow the best care to be provided for the patient and maintain the patient and family in an ideal context.
Child↗
Images in congenital heart disease. Hydropneumopericardium.
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Child↗
Pulmonary artery catheter placement under transoesophageal echocardiography guidance.
Despite correct positioning pulmonary artery catheters may be displaced accidentally. Repositioning may prove to be difficult, especially when high pulmonary artery pressures are present. After failure of usual placement methods, transoesophageal echocardiography was used to guide repositioning of a displaced Swan-Ganz catheter in the early postoperative course after cardiac surgery in a child. This technique proved to be useful in visualizing intracardiac catheter orientation and facilitating pulmonary artery catheter placement at bedside, avoiding transportation of a haemodynamically unstable patient.
Adolescent↗