Evelyn Lechner

Right ventricular to pulmonary artery conduit instead of modified Blalock-Taussig shunt improves postoperative hemodynamics in newborns after the norwood operation

OBJECTIVE Perioperative mortality, prolonged postoperative recovery after the Norwood procedure, and mortality between stage I and stage II might be related to shunt physiology. A right ventricular to pulmonary artery conduit offers a banded physiology in contrast to a Blalock-Taussig shunt. The purpose of this study was to assess the hemodynamic differences and their consequences in the postoperative course between Norwood patients with a Blalock-Taussig shunt and those with a right ventricular to pulmonary artery conduit. METHODS From October 1999 until May 2002, 32 unselected consecutive patients underwent a Norwood procedure at the General Hospital Linz. The first 18 patients received a Blalock-Taussig shunt. In the remaining 14 patients we performed a right ventricular to pulmonary artery conduit. Both groups were compared. RESULTS The diastolic blood pressure was significantly higher in the right ventricular to pulmonary artery conduit group (P <.001). Despite a higher FIO(2), PO(2) levels tended to be lower in the first 5 postoperative days. At the age of 3 months, catheterization laboratory data showed a lower Qp/Qs ratio in the same group (0.86 [0.78; 1] versus 1.55 [1.15; 1.6]; P =.005) and a higher dp/dt (955 [773; 1110] vs 776 [615; 907]; P =.018). (Descriptive data reflect medians and quartiles [in brackets].) Hospital survival was 72% in the Blalock-Taussig shunt group versus 93% in the right ventricular to pulmonary artery conduit group. Mortality between stage I and stage II was 23% in the Blalock-Taussig shunt group versus 0% in the right ventricular to pulmonary artery conduit group. CONCLUSIONS A higher diastolic blood pressure and a lower Qp/Qs ratio were associated with a more stable and efficient circulation in patients with a right ventricular to pulmonary artery conduit. More intensive ventilatory support was necessary during the first postoperative days. We did not note any adverse effects of the ventriculotomy on ventricular performance.

Early primary repair of tetralogy of fallot in neonates and infants less than four months of age.

BACKGROUND The ideal age for correction of tetralogy of Fallot is still under discussion. The aim of this study was to analyze morbidity and mortality in patients who underwent early primary repair of tetralogy of Fallot at the age of less than 4 months and to assess whether neonates, who needed early repair within the first 4 weeks of life, faced an increased risk. METHODS From 1995 to 2006, 90 consecutive patients with tetralogy of Fallot and pulmonary stenosis underwent early primary repair. Patient charts were analyzed retrospectively for two groups: group A, 25 neonates younger than 28 days who needed early operation owing to duct-dependent pulmonary circulation or severe hypoxemia; and group B, 65 infants younger than 4 months of age who underwent elective early repair. RESULTS There was no 30-day mortality; late mortality was 2% after a median follow-up time of 4.7 years. Seven of 88 patients (8%) needed reoperation and twelve of 88 patients (14%) needed reintervention. Groups A and B did not differ significantly in terms of intensive care unit stay, days of mechanical ventilation, overall hospital stay, major or minor complications, or reoperation. Significant differences were found in a more frequent use of a transannular patch (p = 0.045) and more reinterventions (p = 0.046) in group A. CONCLUSIONS Early primary repair of tetralogy of Fallot can be performed safely and effectively in infants younger than 4 months of age and even in neonates younger than 28 days with duct-dependent pulmonary circulation or severe hypoxemia.

Levosimendan versus milrinone in neonates and infants after corrective open-heart surgery: a pilot study.

Objective: Low cardiac output syndrome commonly complicates the postoperative course after open-heart surgery in children. To prevent low cardiac output syndrome, prophylactic administration of milrinone after cardiopulmonary bypass is commonly used in small children. The aim of this study was to compare the effect of prophylactically administered levosimendan and milrinone on cardiac index in neonates and infants after corrective open-heart surgery. Design: Prospective, single-center, double-blind, randomized pilot study. Setting: Tertiary care center, postoperative pediatric cardiac intensive care unit. Patients: After written informed consent, 40 infants undergoing corrective open-heart surgery were included. Interventions: At weaning from cardiopulmonary bypass, either a 24-hr infusion of 0.1 &mgr;g/kg/min levosimendan or of 0.5 &mgr;g/kg/min milrinone were administered. Cardiac output was evaluated at 2, 6, 9, 12, 18, 24, and 48 hrs after cardiopulmonary bypass using a transesophageal Doppler technique (Cardio-QP, Deltex Medical, Chichester, UK). Cardiac index was calculated from cardiac output and the patients’ respective body surface area. Results: Intention-to-treat data of 39 patients (19 in the levosimendan and 20 in the milrinone group) were analyzed using analysis of variance for repeated measurements for statistics. Analysis of variance revealed for both, cardiac index and cardiac output, similar results with no significant differences of the factors group and time. A significant interaction for cardiac output (p = .005) and cardiac index (p = .007) was found, which indicates different time courses of cardiac index in the two groups. Both drugs were well tolerated; no death or serious adverse event occurred. Conclusions: In our small study, postoperative cardiac index over time was similar in patients with prophylactically administered levosimendan and patients with prophylactically given milrinone. We observed an increase in cardiac output and cardiac index over time in the levosimendan group, whereas cardiac output and cardiac index remained stable in the milrinone group. This pilot study has primarily served to obtain experience using the new drug levosimendan in neonates and infants and to initiate further multicenter trials in pediatric patients.

Use of levosimendan, a new inodilator, for postoperative myocardial stunning in a premature neonate.

Objective: To first report the successful use of the new inodilator levosimendan in a premature infant with congestive heart failure (CHF) following cardiac surgery. Although the calcium sensitizer levosimendan improves hemodynamics in adults with CHF, no data are available on the use of levosimendan in premature infants with CHF. Design: Single case report. Setting: Twenty-bed postoperative adult and pediatric cardiac intensive care unit. Patient: A 32 wks gestational age, 1525-g premature male twin with transposition of the great arteries. Interventions: The patient underwent arterial switch operation. Measurements and Main Results: Immediately after operation, the patient developed signs of low cardiac output syndrome. Mixed venous saturation was 56%, serum lactate increased to 14.8 mmol/L, systolic arterial pressure was 40 mm Hg, left atrial pressure was 24 mm Hg, and echocardiography showed reduced left ventricular function with a fractional shortening of 10%. There were no signs of reduced coronary perfusion. Milrinone, dobutamine, and epinephrine did not improve hemodynamics. Levosimendan was initiated at a dose of 0.05 &mgr;g·kg−1·min−1, increased to 0.1 &mgr;g·kg−1·min−1, and continuously infused for 24 hrs. Within 6 hrs after starting the levosimendan infusion, left atrial pressure decreased to 7 mm Hg and systolic arterial pressure increased to 60 mm Hg; within 24 hrs after initiation serum lactate level normalized to 1.7 mmol/L and mixed venous saturation increased to 81%. Echocardiography revealed improvement of left ventricular function with a fractional shortening of 25%. No side effects were recognized during administration of levosimendan. Conclusions: In this premature neonate with postoperative low cardiac output syndrome due to failing myocardial function, levosimendan was a potent inotropic agent.

Open-heart surgery in premature and low-birth-weight infants — a single-centre experience

OBJECTIVE Because of their poor clinical status, infants may require surgery for congenital heart disease regardless of weight or prematurity. This retrospective review describes a single-centre experience with open-heart surgery in low-weight infants. METHODS From November 1997 to December 2006, 411 open-heart surgery procedures were performed in neonates. This included 46 consecutive infants weighing less than 2500 g, who underwent cardiopulmonary bypass for correction of congenital heart defects (n=34) or Norwood stage I palliation of hypoplastic left heart syndrome (HLHS) (n=12). In the low-weight group were 23 males and 23 females with a median age of 10 days and a median weight of 2.26 kg (range: 1.28-2.49 kg). RESULTS Early mortality was 8.2% in patients weighing more than 2.5 kg and 13% in the low-weight group. Within the low-weight group, weight at surgery, history of prematurity and prevalence of additional extracardiac malformations did not influence early mortality. At a median follow-up time of 32 months overall mortality was 21%. Thirty-four patients had a neurological follow-up examination 30 months postoperatively. Of the 34 survivors, 11 showed neurological deficits. CONCLUSIONS In our patient population, early mortality was higher for infants weighing less than 2.5 kg. However, within the low-weight group, lower weight at surgery or history of prematurity was not associated with a higher mortality or bad neurological outcome.

Early and mid-term outcome of the arterial switch operation in 114 consecutive patients : A single centre experience.

BackgroundThe arterial switch operation (ASO) has become the treatment of choice in patients with simple or complex transposition of the great arteries (TGA). The purpose of this study was to assess early and mid-term outcome after ASO in a single centre.Patients and methodsBetween 1995 and December 2005, 114 consecutive patients underwent an ASO at our institution, performed by one single surgeon. Patients charts, surgical reports and echocardiograms were retrospectively reviewed. Patients were analyzed in 3 different groups: Group I consisted of 77 neonates with TGA and intact ventricular septum, group II of 13 patients with TGA and ventricular septal defect which had to be closed surgically and group III of 24 patients with various forms of TGA in a complex setting. The patient’s median weight was 3.23 kg (1.65–8.30). Twenty-five neonates were born preterm, 18 were diagnosed prenatally. Median follow-up time was 20.7 months (0.3–128.6).ResultsThe thirty day mortality was 1.75% (2/114), late mortality 0.88% (1/112) accounting for an overall mortality of 2.63%. There was only one early coronary event and so far no late events. Ten of 111 survivors required reoperation, all but 1 from group III. Prevalence of supravalvular pulmonary stenosis was 4.7%. Freedom from reoperation at 5 years of follow-up time was 87.5%. One patient required permanent pacing, no other late arrhythmias occurred. In our series the only risk factor for increased mortality and morbidity was a body weight of less than 2500 g at the time of operation. No better outcome could be demonstrated in the prenatally diagnosed patients.ConclusionThe ASO can be performed safely and with low mortality and morbidity even in patients with complex TGA. Follow-up of these patients is required to detect residual problems like supravalvular pulmonary stenosis, coronary problems, arrhythmias and aortic valve dysfunction.

Aminoterminal brain-type natriuretic peptide levels correlate with heart failure in patients with bidirectional Glenn anastomosis and with morbidity after the Fontan operation

OBJECTIVE The aims of this study were to generate normal values of aminoterminal pro-brain natriuretic peptide in children with a bidirectional Glenn anastomosis without congestive heart failure and to test the hypothesis that plasma levels of aminoterminal pro-brain natriuretic peptide correlate with the clinical severity of congestive heart failure and morbidity after the Fontan operation. METHODS Aminoterminal pro-brain natriuretic peptide plasma levels of 78 patients after the bidirectional Glenn operation with a median age of 3.2 years and a median follow-up time of 3 years were measured by using an automated enzyme immunoassay. The severity of heart failure was quantified by using the New York University Pediatric Heart Failure Index. RESULTS The 97.5th percentile of aminoterminal pro-brain natriuretic peptide level in patients without congestive heart failure was 339 pg/mL. Aminoterminal pro-brain natriuretic peptide levels strongly correlated with the New York University Pediatric Heart Failure Index score (P < .001). In patients with congestive heart failure (31/78), the aminoterminal pro-brain natriuretic peptide levels were significantly higher (median, 670 pg/mL) than in patients without congestive heart failure (median, 171 pg/mL). In 41 patients who underwent the Fontan operation, the time to removal of chest tubes and the length of hospital stay positively correlated with the preoperative value of aminoterminal pro-brain natriuretic peptide. CONCLUSIONS In children with a bidirectional Glenn anastomosis without signs of heart failure, aminoterminal pro-brain natriuretic peptide levels were within the normal range and correlated with the severity of congestive heart failure. Further studies are needed to determine whether aminoterminal pro-brain natriuretic peptide levels can aide clinicians in the early detection of congestive heart failure in this patient group.

Amino Terminal pro B-Type Natriuretic Peptide Levels Are Elevated in the Cord Blood of Neonates With Congenital Heart Defect

This prospective study investigates whether amino terminal proB-type natriuretic peptide (NT-proBNP) levels are elevated in neonates with congenital heart defect (CHD). NT-proBNP levels in the umbilical cord blood of 60 neonates with prenatally diagnosed CHD, in the cord blood of 200 control subjects, and in the plasma of the respective mothers were analyzed using an automated enzyme immunoassay. NT-proBNP concentrations in the cord blood of the CHD group were significantly elevated compared with controls [median (range): 158 pg/mL (378–3631 pg/mL) and 626 pg/mL (153–2518 pg/mL); p value <0.001]. The NT-proBNP concentrations of the newborns and their mothers did correlate neither in the CHD nor in the control group. In 54 patients with CHD, NT-proBNP levels were measured on the median 5th day of life. They showed a significant increase (median 1665 pg/mL and 19222 pg/mL; p < 0.001). NT-proBNP levels in the cord blood of neonates with CHD are significantly elevated and show a marked increase in the first week of life. Furthermore, this study confirms previously published reference intervals of NT-proBNP in the cord blood of healthy neonates as well as the finding that there is no exchange of NT-proBNP across the placenta.

Tocolysis with the β-2-sympathomimetic hexoprenaline increases occurrence of infantile haemangioma in preterm infants

Background Infantile haemangioma (IH) is the most commonly observed tumour in children. Off-label pharmacological treatment of IH with the beta-blocker propranolol induces regression of IH. Based on the fact that IH are more frequently observed in premature babies than in mature babies and the evidence that beta-blocker therapy leads to regression of IH, the authors generated the hypothesis that the use of β-2-sympathomimetics during pregnancy for inhibiting premature labour might increase occurrence of IH in preterm infants. Methods For group comparison t test, Mann–Whitney U test and Fishers exact test were used. Logistic regression was carried out by the forward stepwise method with Wald statistics. Results Data of 328 preterm infants (<32 gestational weeks) or with a birth weight of less than 1500 g (<36 gestational weeks) born between January 2006 and December 2008 were analysed. A total of 15 were excluded due do death within the 1st month of life, 38 because of lost to follow-up and six due to incomplete data. Complete data of 269 preterm infants were retrospectively analysed. During the follow-up period of median 1.6 years, 50 infants developed one or more IH within their first 6 months of life. IH occurred in 40/181 patients with intrauterine exposure to the β-2-sympathomimetic hexoprenaline and in 10/88 without exposure (OR=4.3; 95% CI 1.4 to 13.8). Furthermore, the influence of antenatal exposure to glucocorticosteroids for induction of lung development was analysed. Prenatally exposed subjects showed reduced occurrence of IH (OR=0.2; 95% CI 0.05 to 0.8). Conclusion Intrauterine exposure to the β-2-sympathomimetic hexoprenaline might increase the occurrence of IH in preterm infants.

Facial Nerve Paralysis in Children: Is It as Benign as Supposed?

BACKGROUND Facial nerve paralysis is a common disease in children. Most of the patients show complete recovery. This single-center cohort study exclusively included pediatric patients to investigate the outcome of all patients with facial nerve palsy. METHODS Hospital records of all the patients admitted to the Childrens Hospital in Linz between January 2005 and December 2010 with facial nerve paralysis were reviewed. Patients with peripheral facial nerve palsy were invited for clinical reevaluation between July 2011 and October 2011. The House-Brackmann score was used for reassessment. RESULTS Fifty-six patients agreed to return for an additional clinical reevaluation. Study participants were divided in two groups according to their House-Brackmann scores: group 1 (n = 44), with a score <2 were considered good outcomes, and group 2 (n = 12), with a score ≥ 2 showed persistent mild to moderate dysfunction of the facial nerve and were considered moderate outcomes. The most important finding was the difference of the reported time to remission (P = 0.003) between the groups. CONCLUSION The results of this study indicate that facial paralysis in children is not as benign as supposed. It is suggested that patients and their guardians be informed that a slight face asymmetry may persist, but functional recovery in general is excellent.