Giovanna Boccuzzo

Scimitar Syndrome A European Congenital Heart Surgeons Association (ECHSA) Multicentric Study

Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used. # Clinical Perspective {#article-title-44}Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.

Is there an optimal timing for surgical ligation of patent ductus arteriosus in preterm infants

BACKGROUND We sought to define the variables associated with hospital outcome in preterm infants with patent ductus arteriosus (PDA) and identify the optimal timing for PDA closure to improve hospital outcome. METHODS Included were 201 premature babies (< or = 32 weeks gestational age), from January 2001 to June 2007, with PDA who received primary medical treatment with ibuprofen. Number of ibuprofen cycles, gestational age, body weight, and presence of symptomatic hypotension requiring vasoactive/inotropic drugs were related to hospital outcome, including hospital mortality, presence of necrotizing enterocolitis, acute renal failure, intraventricular hemorrhage, retinopathy and bronchopulmonary dysplasia at week 36. Data were analyzed with a logistic regression model. RESULTS Medical treatment was effective in 149 patients (75%), but 52 (25%) required surgical ligation after medical treatment failed. They had younger gestational age (25 weeks [IQR, 24 to 27 weeks] vs 27 weeks [IQR, 25 to 28 weeks], p < 0.0001), lower body weight at birth (730 g [IQR, 595 to 915 g] vs 840 g [IQR, 670 to 1016], p = 0.05), and a higher incidence of symptomatic hypotension (38 of 52 [73%] vs 56 of 149 [38%], p < 0.0001) than patients who responded to ibuprofen. More than two cycles of ibuprofen was significantly associated with an increased risk for bronchopulmonary dysplasia (odds ratio [OR], 2.81; p = 0.03) and acute renal failure (OR, 3.81; p = 0.09). CONCLUSIONS The prolonged patency of the ductus arteriosus in preterm infants is related to an increased morbidity. Surgical ligation of PDA is a safe and effective treatment and should be done soon after two complete cycles of ibuprofen, especially in selected patients, to improve clinical outcome.

Remifentanil for percutaneous intravenous central catheter placement in preterm infant: a randomized controlled trial

Background:  There is limited evidence on the analgesic efficacy of opioids during percutaneous intravenous central catheter (PICC) insertion in preterm infants.

Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study

Background— To evaluate indications and results of surgery for primary cardiac tumors in children. Methods and Results— Eighty-nine patients aged ≤18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate ( P= 0.006). Overall mortality was associated to malignancy ( P =0.0008), and adverse events during follow-up ( P =0.005). Conclusions— Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas. # Clinical Perspective {#article-title-51}Background— To evaluate indications and results of surgery for primary cardiac tumors in children. Methods and Results— Eighty-nine patients aged ⩽18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate (P=0.006). Overall mortality was associated to malignancy (P=0.0008), and adverse events during follow-up (P=0.005). Conclusions— Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas.

Surgical repair of congenital mitral valve malformations in infancy and childhood: A single-center 36-year experience

OBJECTIVE We sought to evaluate the results of surgical repair and determine predictors for the late outcome of congenital mitral valve dysplasia. METHODS Preoperative, operative and postoperative data were obtained from an institutional database; follow-up data came from regular clinical evaluation at our institution or elsewhere. Patients were divided into isolated and complex cases according to the complexity of associated lesions. RESULTS Between 1972 and 2008, 93 patients (43 male and 50 female patients) underwent mitral repair (median, 4.5 years; range, 0.16-19.8 years). Predominant mitral regurgitation was present in 52%. Associated cardiac anomalies were present in 72%. Sixty-one patients were in the complex group. All patients underwent successful mitral repair. Surgical repair was tailored to the patients valve anatomy. Early death was 7.5%. The postoperative course was uneventful in 86% of patients. At a mean follow-up of 10.3 years (median, 8.4 years; completeness, 94%), late mortality is 8% (7 patients). Twelve patients underwent mitral reintervention (11 replacements and 1 repair). Among the 80 survivors, 82.5% were in New York Heart Association class I or II, and 61.2% had some degree of persistent mitral regurgitation or stenosis, despite stable hemodynamics. Stenosis is a statistically significant risk factor for surgical intervention at less than 1 year of age and is related to higher overall mortality and incidence of late cardiac failure and mitral dysfunction; parachute mitral valve is related to higher mortality and morbidity. CONCLUSIONS Mitral valve repair shows acceptable early mortality and reoperation rates. Mitral malformations in the complex group are related to a significantly higher risk of reoperation on the mitral valve. Parachute mitral valve is associated with a higher rate of early mortality.

Pain management during invasive procedures at Italian NICUs: has anything changed in the last five years?

Objective: To ascertain the extent to which neonatal analgesia for invasive procedures has changed in the last 5 years since the publication of Italian guidelines. Methods: We compared survey data for the years 2004 and 2010 on analgesia policy and practices for common invasive procedures at Italian Neonatal Intensive Care Units (NICUs); 75 NICUs answered questionnaires for both years and formed the object of this analysis. Results: By 2010 analgesia practices for procedural pain had improved significantly for almost all invasive procedures (p < 0.05), both non-pharmacological and pharmacological methods being adopted by the majority of NICUs (unlike the situation in 2004). The routine use of medication for major invasive procedures was still limited, however (35% of lumbar punctures, 40% of tracheal intubations, 46% during mechanical ventilation). Postoperative pain treatment was still inadequate, and 41% of facilities caring for patients after surgery did not treat pain routinely. Pain monitoring had definitely improved since 2004 (p < 0.05), but not enough: only 21% and 17% of NICUs routinely assess pain during mechanical ventilation and after surgery, respectively. Conclusion: There have been improvements in neonatal analgesia practices in Italy since national guidelines were published, but pain is still undertreated and underscored, especially during major invasive procedures. It is mandatory to address the gap between the recommendations in the guidelines and clinical practice must be addressed through with effective quality improvement initiatives.

The Evolution of the Right Anterolateral Thoracotomy Technique for Correction of Atrial Septal Defects: Cosmetic and Functional Results in Prepubescent Patients

BACKGROUND The aim of this study was to evaluate the results of a cosmetic right anterolateral thoracotomy (RALT) in prepubescent girls who underwent repair of atrial septal defect (ASD). METHODS All prepubescent girls who underwent RALT for ASD closure since January 1998 were included. Patient satisfaction with the cosmetic approach and the impact of RALT on breast development were evaluated. One hundred healthy women were used as controls. RESULTS One-hundred forty-one patients were included. Median age at repair was 4.6 years (range, 8 months-12 years). The median length of the surgical incision was 9 cm (range 3-20 cm); it was shorter during the last 5 years of our experience (median, 5 cm; range, 3-10 cm; p = 0.001). Fourteen patients (9.9%) experienced a transitory mild sensitive skin deficit in the mammary area, which was associated with the length of the RALT (p = 0.05). At median follow-up of 10.1 years (range, 1.5-14.5 years), 3/60 patients (5%) who had complete breast development had relevant asymmetrical breast development (indexes of breast symmetry > 2 standard deviations of the median value for controls). Six additional patients who underwent RALT (10%) had mild asymmetrical breast development, which was comparable to the control population (p = 0.1). The satisfaction rate for the cosmetic result of RALT was 97.8% (138/141 patients). Reasons for dissatisfaction were the presence of asymmetrical breast development (n = 2) and the presence of a keloid at the incision level (n = 1). CONCLUSIONS RALT is a reliable surgical technique that combines excellent cosmetic and functional results in almost all patients. With the latest technical refinements, it is our gold standard treatment for ASD in prepubescent girls.

Procedural pain in neonates: the state of the art in the implementation of national guidelines in Italy

National and international guidelines have been published on pain control and prevention in the newborn, but data on compliance with these guidelines are lacking.

Near-infrared spectroscopy for monitoring leg perfusion during minimally invasive surgery for patients with congenital heart defects.

Regional oxygen saturation measured by means of nearinfrared spectroscopy (NIRS) have been used as potential surrogate of cerebral and somatic mixed venous oxygen saturation and has been suggested as a noninvasive tool to continuously monitor and detect states of low body perfusion. We sought to evaluate the safety of peripheral arterial cannulation by using NIRS in patients undergoing minimally invasive heart surgery for correcting simple congenital heart diseases, focusing on patients with a body weight less than 30 kg.

Assessing the need for computed tomography for lower-third-molar extraction: a survey among 322 dentists

PurposeOrthopantomograms (OPT) are used to assess the anatomical relationship between the inferior alveolar nerves (IAN) and the roots of third molars and the related risk of postextraction iatrogenic neurological lesions. When the risk is high, computed tomography (CT) or conebeam CT may be warranted. We investigated how dentists judged the need for CT from OPT to ascertain whether they comply with criteria of justification, appropriateness and optimisation in prescribing examinations involving radiation.Materials and methodsA total of 2,713 letters were sent to Italian dentists (Veneto region), inviting them to access an Internet Web site showing 20 OPTs and answer a questionnaire on the need for CT or periapical X-ray. The gold standards were CT images corresponding to the OPTs. The respondents’ answers were rated for appropriateness and their tendency to over- or underprescribe CT.ResultsThe questionnaire was completed by 11.9% of the dentists contacted. The response rate was compatible with a Web survey. Their answers generally came close to the gold standard, achieving a mean appropriateness rating of 0.636 (range 0–1). An overlap between the mandibular canal and the third-molar root was the anatomical relationship most often noted. Recommendations for CT were proportional to the number of radiographic signs indicating a risk of inferior alveolar nerve injury. Periapical X-ray was considered useful by 54.9% of dentists not recommending CT. The main reason stated for not recommending CT was that it was unnecessary for the purposes of the extraction.ConclusionsOur survey revealed a cautious approach among the professionals interviewed, who tended to overprescribe CT.RiassuntoObiettivoL’ortopantomografia (OPT) è utilizzata per valutare la vicinanza del nervo alveolare inferiore (NAI) alle radici del terzo molare e il rischio di una lesione neurologica iatrogena post-estrattiva. Quando il rischio è elevato, può essere richiesta la tomografia computerizzata (TC) o TC cone beam. Lo scopo del nostro lavoro è di verificare come viene percepita la necessità di TC dopo visione di OPT da parte di un campione di dentisti, ovvero se vi è rispetto dei criteri di giustificazione, appropriatezza e ottimizzazione nella prescrizione di esami radiologici.Materiali e metodiSono state inviate 2713 lettere a dentisti italiani (regione Veneto), chiedendo di accedere ad un sito internet contenente 20 OPT ed un questionario riguardanti l’indicazione alla TC o alla radiografia endorale. Come gold standard si sono utilizzate le TC corrispondenti alle OPT. Le risposte degli intervistati sono state valutate per correttezza e tendenza a prescrivere o non prescrivere la TC.RisultatiHa risposto l’11,9% della popolazione oggetto d’indagine. Il tasso di risposta è compatibile con un sondaggio web. I rispondenti si sono avvicinati al gold standard e l’indice di correttezza totale è stato pari a 0,636 (range di 0–1). La sovrapposizione del canale mandibolare e della radice è la relazione più rilevata. La richiesta di TC è proporzionale al numero di segni rilevati. Il 54,9% di chi non prescrive la TC ritiene utile una radiografia periapicale. Il motivo principale per cui non viene prescritta la TC è per la mancanza di necessità ai fini dell’estrazione.ConclusioniIl nostro studio ha rivelato una certa cautela dei professionisti che tendono a sovra-prescrivere la TC.