Body mass index stratification in hospitalized Italian adults with congenital heart disease in relation to complexity, diagnosis, sex and age.

BACKGROUND AND AIMS: Adults with congenital heart disease (ACHD) are at risk of overweight and obesity, two major health problems, though underweight can be a negative prognostic factor too. Awareness of the body mass index (BMI) in ACHD is very limited. The present study describes the use and prevalence of BMI in Italian symptomatic hospitalized ACHD patients in relation to complexity by Bethesda system classification, diagnosis, sex and age. METHODS AND RESULTS: We classified 1388 ACHD patients, aged 18-69 years, on the basis of their BMI, and compared them to the Italian reference population. In our total ACHD population we found a significantly higher prevalence of underweight compared to the Italian reference population (6.34% vs 3.20%). ACHD women were more underweight than men. Underweight decreased with age. Overweight was significantly less frequent in the total ACHD population (26.73% compared to 31.70%) in the Italian reference population. Men were more likely to be overweight than women. In statistical terms obesity was similar in the Italian reference population (10.50%) and our ACHD population (9.58%). Both overweight and obesity increased with age. Results were comparable using a diagnostic anatomical-functional classification and the Bethesda system classification. CONCLUSIONS: In our cohort of ACHD the prevalence of underweight was double that of the Italian reference population. The prevalence of overweight was lower, while obesity was similar. Since BMI does not account for differences in body fat distribution, a future aim will be to quantify the visceral component of the adipose tissue in ACHD patients and examine their body composition in order to reflect their risk of acquired cardiovascular disease better, and either to maintain or achieve an adequate visceral component.

Early or late fresh frozen plasma administration in newborns and small infants undergoing cardiac surgery: the APPEAR randomized trial.

BACKGROUND: In newborns and small infants undergoing cardiac surgery with cardiopulmonary bypass (CPB) and blood priming, it is unclear whether there is reduced blood loss if fresh frozen plasma (FFP) is added to the CPB priming volume. This single-centre, randomized trial tested the hypothesis that the administration of FFP after CPB (late FFP group) is superior to FFP priming (early FFP group) in terms of postoperative bleeding and overall red blood cell (RBC) transfusion. METHODS: Seventy-three infants weighing <10 kg were randomly allocated to receive FFP to supplement RBCs in the CPB priming solution ( n =36) or immediately after CPB ( n =37). The primary endpoint was a difference in postoperative blood loss; secondary endpoints included the amount of RBCs and FFP transfused through the first 48 postoperative hours. RESULTS: All patients were included in the analysis. Patients in the late FFP arm had greater postoperative mean blood loss than patients in the early FFP arm [33.1 ( sd 20.6) vs 24.1 (12.9) ml kg -1 ; P =0.028], but no differences in transfusions were found. The subgroup of cyanotic heart disease patients had comparable results, but with greater use of RBCs in the late FFP group. CONCLUSIONS: In infants undergoing cardiac surgery, FFP in the priming solution appears slightly superior to late administration in terms of postoperative bleeding. CLINICAL TRIAL REGISTRATION: www.ClinicalTrials.gov , NCT02738190.

Life experiences and coping strategies in adults with congenital heart disease.

Many adults with congenital heart disease (ACHD) have to face considerable psychosocial difficulties. The aim of this study was to explore the life experiences of ACHD patients, from when they become aware of having a condition, till after the open heart surgery they underwent. The study was conducted with the use of unstructured, in-depth interviews, performed on 11 patients (age ranging: 20 - 56 y) after they recovered from open heart surgery and a focus group, which included 16 participants (age ranging: 22 - 46 y). Both the interviews and the focus group were recorded, transcribed and analyzed according to Grounded Theory procedures. Our findings show that the condition of diversity is the core of the emotional experiences connected to ACHD. Feeling different and being perceived as being different are clearly interlinked and coping strategies adopted resulted as being influenced by this perception. This study also clearly outlines the importance of having an adequate perception of one's condition and the link between maladaptive coping strategies and an incorrect perception of one's heart condition. Results are discussed in order to promote psychosocial interventions within and outside of the hospital setting in order to improve the patients' emotional wellbeing.

Pattern of cerebral ultrasound in neonatal heart surgery.

AIM: No study described reliably the changes in cerebral ultrasound (CUS) findings in neonatal heart surgery. We tried to define the modifications of CUS before and after heart surgery in neonates. PATIENTS: We studied 48 neonates with congenital heart defects were studied. Of these, 33 had correction of the malformation with cardiopulmonary bypass (CPB group); 15 underwent coarctation repair/shunting procedures (no-CPB group). Patients had CUS pre-operatively, 48-72 hours after surgery, and at discharge. RESULTS: Pre-operative studies did not show significant differences between the groups. In CPB group, preoperatively, 6/33 infants showed echogenicity of basal ganglia and 1/33 grade I IVH. In the early postoperative evaluation, 24/33 infants had abnormal scans: increased echogenicity of basal ganglia in 24 and grade I IVH in 14. At discharge, abnormal scans persisted in 20/33 (basal ganglia echogenicity in 20, grade I IVH in 12). In the no-CPB group, preoperatively, 1/15 infant showed echogenicity of basal ganglia. In the early postoperative evaluation, 6/15 had abnormal scans: increased echogenicity of basal ganglia in 6, grade I IVH in 4. At discharge, abnormal scans persisted in 3/15. CONCLUSIONS: CUS after neonatal heart surgery shows a definite pattern of increased echogenicity of the basal ganglia, more consistent after CPB. These changes may reflect disturbances of deep grey matter, and last 10-15 days at least.

Late surgical treatment of tetralogy of Fallot.

AIM: To study early post-operative results and follow up of patients over a year old, operated on for tetralogy of Fallot (ToF). METHODS: This retrospective analysis included 22 patients (14 male and eight female) with a mean age of 9.18 ± 6.5 years (range 13.5 months to 26 years), who underwent complete repair of ToF between April 2003 and June 2009. Data from patients' records, pre-operative cardiac catheterisation studies, operative intervention, and pre-operative and postoperative two-dimensional echocardiographic studies were reviewed. All patients underwent complete repair including closure of ventricular septal defect (VSD). A trans-annular patch was used in 12 patients while an infundibular patch was used in 10 others. Patients were evaluated one, three, six and 12 months after surgery, and annually thereafter. The duration of follow up was from eight months to six years post surgery. RESULTS: Classical ToF was found in 10 patients. Twelve cases had associated anomalies: two patients with hypoplastic pulmonary artery branches, two with arterial duct malformations, and eight had proximal stenosis of the left branch of the pulmonary artery. NHYA class distribution was as follows: class I: two patients; class II: five subjects; class III: 10 patients; class IV: five subjects. The mean stay in hospital was 15 ± 7 days. Two patients (9%) died during the early post-surgical period. At a mean follow-up interval of 32 ± 9 months, all patients were asymptomatic and in NYHA class I. No late deaths occurred. In three patients, we registered isolated monomorphic ventricular extrasystoles. The right ventricle outflow tract (RVOT) pressure gradient was 29 ± 1.5 mmHg in the acute post-surgical period and it did not change significantly during follow up. The right ventricular function was defined as normal in 95% of the patients in the study and was mildly depressed in 5%. CONCLUSION: Even if treated later in life, our study showed very good surgical results of patients with ToF.

Occurrence and pattern of congenital heart diseases in a rural area of sub-Saharan Africa.

UNLABELLED: The extent of congenital heart disease in Cameroon remains largely unknown. The aim of this study was to determine the occurrence and pattern of congenital heart diseases in the Cardiac Centre of St Elizabeth Catholic General Hospital, situated in a rural area of Cameroon. METHODS: Between November 2002 and November 2008, a population of 2 123 patients with suspected cardiac pathologies were consulted at St Elizabeth Catholic General Hospital referral cardiac centre. Of these patients, 292 subjects were recruited for the study, based on detection of (1) precordial murmurs and/or cardiomegaly on chest X-ray examination, or (2) congenital heart diseases on transthoracic Doppler echocardiography examination. RESULTS: Congenital heart diseases and inorganic murmurs were found in 95.5 and 4.5% of the patients, respectively. Congenital heart diseases included tetralogy of Fallot (26.1%), isolated ventricular septal defect (38.8%), atrioventricular cushion defect (7.3%), isolated atrial septal defect (2.8%), arterial duct cases (12.4%), common arterial trunk (1.3%), isolated stenosis of the pulmonary artery (2.6%), coarctation of the aorta (1.1%), congenital mitral valve regurgitation (1.2%), atresia of the triscupid valve (1.6%), double-outlet right ventricle (2.1%), anomalous pulmonary venous return (1.5%) and left isomerism (1.2%). CONCLUSION: Our data show that there is a high occurrence of congenital heart disease in this hospital in a rural zone of sub-Saharan Africa and that isolated ventricular septal defect is the predominant pathology. Post-surgical follow up remains very challenging as many parents cannot afford their children's medical treatment or are generally not well educated.

Pattern and clinical aspects of congenital heart diseases and their management in Cameroon.

UNLABELLED: The aim of the study was to investigate the occurrence, pattern and clinical aspects of congenital heart diseases and their management in Cameroon. In this multicentred retrospective study from January 2006 till November 2009, out of 51,636 consulting in three referral centres, 505 were recruited. All the patients presented with the following symptoms: cyanosis, clubbing, frequent respiratory tract infections, failure to thrive, growth retardation, precordial murmur and dyspnoea. Patients were sent for the screening of congenital heart disease. After the comprehensive Doppler echocardiography, the recruited patients were diagnosed with congenital (67.2%) and in few, acquired heart disease. Heart murmur, dyspnoea and growth retardation was the triad mostly encountered. The occurrence of congenital heart diseases in Cameroon is 9.87%. In Douala, isolated ventricular septal defect, interatrial septal defect and isolated pulmonary valve stenosis were more diagnosed than in Shisong (P <0.05) and Yaoundé (P <0.05). In Yaoundé, there were more cases of common arterial trunk, transposition of great arteries with ventricular septal defect and Ebstein disease than in Shisong (P <0.05) and Douala (P < 0.05). At Shisong, tetralogy of Fallot, arterial duct, coarctation of the aorta, congenital mitral valve regurgitation, atresia of the tricuspid valve, double outlet right ventricle, anomalous pulmonary venous return and left isomerism were more diagnosed than in Yaoundé (P <0.05) and in Douala (P <0.05). Thirty percent of the patients were operated abroad; 9% in the cardiac centre. CONCLUSION: Our data show that congenital heart diseases are represented in Cameroon as in the literature; isolated ventricular septal defect is the predominant pathology.

Postoperative nutrition of neonates undergoing heart surgery.

OBJECTIVE: Postoperative nutrition of newborns undergoing heart surgery (HS) is ill-defined. We compared the postoperative growth rates (grams/day) of neonates with congenital heart defects (CHDs) fed with their own mothers' milk (HU), a starting formula (SF; 67 kcal/100 mL), or a "preterm formula" (PF; 80 kcal/100 mL). PATIENTS AND METHODS: We studied 122 newborns undergoing HS: 81 underwent corrective surgery (group A), and 41 palliative surgery (group B). RESULTS: No statistically significant differences were found in the growth rate between group A and B. Moreover, in both groups, no differences in terms of growth rate between infants fed HU, SF, or PF were observed. This was also true when analyses were limited to infants undergoing HS either with or without cardiopulmonary bypass. CONCLUSIONS: In neonates undergoing HS, human milk allowed a growth rate similar to that observed with starting formulas and "preterm formulas". This effect may depend on the particular features of human milk and its protective properties for the intestinal mucosa.

[Adult with congenital heart disease: an overview]. / L'adulto con cardiopatia congenita--introduzione.

The improvement of surgical procedures over the past 40 years has changed the medical history of the congenital heart defect. It is estimated that 85% of children with congenital heart disease now have the opportunity to reach adulthood and that this percentage will increase over the next two decades. This special issue of the Journal aims to offer a basic information on key topics of this field of cardiology not newer, but still rapidly evolving.

[Surgical approach to adult with congenital heart disease]. / Trattamento chirurgico delle cardiopatie congenite nel paziente adulto.

The number of adults with congenital heart disease will inevitably increase in the near future. Recent data suggest that the number of adults with congenital heart disease, whether repaired or not, approaches the number of children with the disorder. Cardiac surgery in these patients presents major difficulties in management and technique. This especially applies to cardiac reoperation, which is one of the most frequent interventions and the most common cause of death in these patients. The present study reviews our experience with a large population of 811 consecutive patients who underwent cardiac operation or reoperation between January 2000 to December 2009 at our Institution.

[Right ventricular restoration in adult patients after TOF surgery]. / Rimodellamento del ventricolo destro nel paziente Fallot adulto corretto.

OBJECTIVE: Pulmonary regurgitation may cause progressive right ventricular dilatation and dysfunction in adult patients previously repaired for tetralogy of Fallot. To assess the optimal surgical timing, the impact of the right ventricular restoration with a new surgical ventriculoplasty technique is evaluated following TFO repaired adult patients with severe pulmonary regurgitation and right ventricular dilatation. METHODS: Sixteen patients with severe pulmonary valve regurgitation (PVR) and right ventricular dilatation with RVOT aneurysm underwent right ventricular remodelling since January 2002. Each underwent preoperative evaluation by Doppler echocardiography, magnetic resonance imaging (MRI), and right ventricular myocardial acceleration during isovolumic contraction (IVC). The surgical procedure included pulmonary valve implantation and RVOT restoration achieved by removal of the aneurysm tissue, coupled with a ventriculoplasty to reduce volume, accomplished by creating a satisfactory RVOT dimension by placing with 2-0 Gortex suture to allow acceptance of a 26 Hegar dilator to avoid restriction. Nine patients had associated surgical procedures. RESULTS: All patients survived the operative procedure and underwent a 16-month follow-up interval. A reduction of cardio thoracic index and a clinical improvement occurred in each patient. Significant reduction of RVEDV and RVESV and increased right ventricular ejection fraction was observed. CONCLUSIONS: This preliminary database implies that the right ventricular restoration is a simple and effective procedure, and introduces a structural component that should be added in repaired TFO patients with right ventricular dilatation and underlying aneurysm or akinesia of the right ventricular outflow tract.

[Tetralogy of fallot: evolution of medical-surgical management (part 1)]. / Tetralogia di Fallot: evoluzione del management medico-chirurgico (Parte prima).

Despite Tetralogy of Fallot is a well-known cardiac congenital disease, still an important cardiovascular surgery and intensive care challenge. The following is a selective account of medical and surgical concepts and procedures; the emphasis is on therapeutic developments during the last 50 years. This review is divided into two parts: the first of which traces the evolution of medical and palliative techniques; the second part approaches the surgical corrections and try to explain the reinterventional causes and the residual defects responsible of cardiac failure.

[Tetralogy of Fallot: evolution of medical-surgical management (part 2)]. / Tetralogia di Fallot: evoluzioni del management medico-chirurgico (parte seconda).

This review is divided into two parts: the first of which traces the evolution of medical and palliative techniques; the following is a selective account of surgical concepts and procedures; the emphasis is on therapeutic developments during the last 50 years. The second part approaches the surgical corrections and tries to explain the reinterventional causes and the residual defects responsible of cardiac failure.

Octreotide in the management of postoperative chylothorax.

Chylothorax (KT) may be a complication of thoracic surgery. Its management is not well established and may comprise dietary interventions and surgery. The effectiveness of somatostatin and its analogues has been reported, although their mechanism(s) of action is unclear. We report our experience with octreotide in a series of patients with postoperative chylothorax. Eight patients with KT were treated with a continuous intravenous infusion of octreotide (OCT) at a starting dose of 0.5 microg/kg/hr. They were compared with four additional patients with KT who were treated according to the conventional approach. After a mean of 3.3 +/- 1.9 days of treatment, fluid discharge diminished dramatically. In all patients, fluid losses stopped by postoperative day 10.5 +/- 2.9 and chest tubes could be removed after 12.8 +/- 4.1 days. Compared to a small group of historical controls, OCT reduced significantly the total fluid losses (141.1 +/- 89.3 vs 396.7 +/- 151.0 ml/kg; p = 0.003) and the postoperative length of stay (p = 0.05). No patients in the group treated with OCT required parenteral nutrition (compared to all four of the controls; p = 0.002) and/or thoracenteses (compared to two of four controls). In postoperative KT, OCT seems to be at least as effective as the conventional approach. Furthermore, OCT may reduce total fluid losses and postoperative length of stay. This may have a beneficial effect on the risk of complications and on hospital costs.

Association of Children with Heart Disease in the World: 10-year experience.

Thousands of children live in developing countries with untreated but correctable congenital heart disease (CHD), and most of them will die simple because they live in poor countries that do not have suitable medical/surgical facilities. The aim of the Association of Children with Heart Disease in the World is to support the operatory and teaching activities of a group of voluntary doctors engaged in the management of children with CHD in developing countries. Since 1992, the association has obtained 160 grants for foreign doctors who have been trained in our hospitals; performed 75 missions abroad, with more than 2150 cases evaluated and 404 cardiac operations performed; obtained millions of dollars worth of donated medical equipment; and constructed two cardiac surgery departments in Syria and Cameroon. Many efforts have been made, but much more can be done as we attain more experience and develop better coordination and collaboration between the international groups engaged in this emerging social need.

Growth after neonatal arterial switch operation for D-transposition of the great arteries.

The objective of this study was to evaluate growth in infants with d-transposition of the great arteries (d-TGA) who underwent switch operation (anatomical correction) in the early neonatal period. Growth data (at birth and 1, 3, 6, 12, and 24 months of age) were retrieved in 23 infants with d-TGA who survived the operation without major complications. Measures were transformed into z scores and compared with normative data for age. Following surgery, infants showed low z scores for weight, height, and head circumference. Weight and height showed a trend to reach normal ranges between 6 and 12 months of age, and the z scores were positive at 24 months. z scores for head circumference were still negative at 24 months of age. No dietary interventions were implemented during this period, and the infants were fed their own mothers' milk or, before discharge, high-calorie, preterm formula. Following anatomical correction for d-TGA in the early neonatal period, infant growth shows a characteristic pattern, with parameters normalized by 12 months of age in most cases. Head growth shows a different pattern, although gross motor development seems unaffected.

Atrioventricular septal defect with 'absent' pulmonary valve in the setting of Down's syndrome: a rare association.

We report a rare case of a 2-year-old boy with Down's syndrome, atrioventricular septal defect and so-called 'absent pulmonary valve syndrome'. Diagnostic imaging also revealed the presence of an anomalous high origin of the right coronary artery from the ascending aorta. Surgical repair was successful.

Extended end-to-end anastomosis with modified reverse subclavian flap angioplasty.

We report a surgical treatment for neonatal aortic coarctatin associated with distal aortic arch hypoplasia. This technique offers the possibility for augmentation of the aortic arch without sacrificing the subclavian artery or using prosthetic patch material. The procedure was successfully performed in 5 patients.