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 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.

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.

[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.

[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.

[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.

Pitfalls in echocardiographic-based repair of aortic coarctation.

The knowledge of echocardiographic pitfalls, which may impair the correct indication for surgery of aortic coarctation, is of great importance to pediatric cardiologists. We believe that only the suspicion of coronary artery anomalies is a clear indication to perform a cardiac catherization.

Airway obstructions associated with congenital heart disease in infancy.

Significant airway obstruction may complicate the natural and surgical history of infants with congenital heart diseases. Airway obstruction occurred in 12 infants who had operations for congenital heart disease. In all cases tracheography demonstrated the cause of airway obstruction. Significant mortality (5/12, 41.7%) and morbidity in this group of infants were the result of airway obstruction. To reduce the complications caused by airway obstruction in infants with congenital heart disease, we recommend (1) preoperative identification of patients with potential airway obstruction, (2) preoperative tracheography in high-risk infants, (3) appropriate choice of the surgical procedure, especially when insertion of a prosthetic conduit is required, (4) early recognition of the problem during the postoperative period, (5) prompt diagnosis by postoperative tracheography, with or without angiography, and (6) therapy directed at the cause of airway obstruction.

Tricuspid valve avulsion or excision for right ventricular decompression in pulmonary atresia with intact ventricular septum.

Retrograde right ventricular decompression through the tricuspid valve, by damaging or excising the valve, was attempted in five patients with pulmonary atresia, intact ventricular septum, and severely hypoplastic right ventricle. Two patients were neonates in critical condition, one of whom had received previous palliation with a shunt; the other neonate had received pulmonary valvotomy plus shunt followed by repeat valvotomy plus shunt ligation. Three patients were infants or children who had undergone placement of isolated neonatal shunts. One procedure was performed during cardiac catheterization. Three patients survived. In one, tricuspid avulsion and a Rashkind septostomy were done by percutaneous methods; in two patients, tricuspid excision plus bidirectional cavopulmonary anastomosis was done by open operation. At restudy, all showed subsystemic pressure in the right ventricle and diminution or disappearance of sinusoids. One also showed improvement of left ventricular function. Incorporation of a small right ventricle in the right atrium at the time of the two orthoterminal palliations seemed, at least, innocuous. Right ventricular decompression by tricuspid avulsion or excision could be suggested for all patients with pulmonary atresia, intact ventricular septum, hypersystemic hypoplastic right ventricle, and major sinusoids without right ventricular dependent coronary circulation in whom the outlet portion of the right ventricle is not functional.

Total anomalous pulmonary venous connection: surgical repair with a double-patch technique.

A double-patch technique has been used to repair total anomalous pulmonary venous connection in 35 patients (mean age, 4.9 months; mean weight, 4.2 kg). In all patients with supracardiac or subdiaphragmatic type of total anomalous pulmonary venous connection, repair has been accomplished by enlarging the "new" left atrium. There were three early deaths (3 of 35 = 8.6%) and no late deaths in a mean follow-up of 30 months (3 to 71 months). We propose this double-patch technique as an alternative surgical option for total anomalous pulmonary venous connection.

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.

Neonatal pulmonary autograft implantation for cardiac tumor involving aortic valve.

We diagnosed in a 4-day-old neonate a cardiac tumor involving the left atrium, left atrioventricular junction, left ventricular outflow tract, and aortic valve with severe subvalvular and valvular aortic stenosis. The critical involvement of the aortic valve and the scarcity of neonatal cardiac donors led us to perform a successful replacement of the aortic root with a pulmonary autograft, using a very small homograft for the native pulmonary valve (Ross operation).

Total cavopulmonary direct anastomosis: a logical approach in selected patients.

A 2.5-year-old boy with a diagnosis of situs solitus, tricuspid atresia, anatomically corrected malposition of the great arteries (S,D,L), left juxtaposition of atrial appendages, and pulmonary stenosis underwent successful total cava-to-pulmonary connection by means of a superior vena cava-to-pulmonary artery end-to-side anastomosis associated with an inferior vena cava-to-pulmonary artery direct anastomosis. Anatomic features and surgical technique are described.

Partial atrioventricular canal with congestive heart failure in the first year of life: surgical options.

BACKGROUND: An important subgroup of patients with partial atrioventricular canal require an operation in the first year of life because of refractory congestive heart failure. METHODS: From June 1982 to April 1995, of 128 patients with partial atrioventricular canal, 35 patients (27%) underwent surgical treatment at less than 1 year of life. Associated cardiac anomalies were present in 22 patients. Only 7 patients (20%) had Down's syndrome. Five patients with left ventricular hypoplasia underwent aortic coarctectomy (3 patients) or Norwood operation (2 patients). The other 30 patients underwent anatomic repair in 24 cases and aortic coarctectomy in 6. The surgical results of patients submitted for anatomic repair were retrospectively correlated with the echocardiographic mitral valve diameter. RESULTS: There were 7 deaths (29%) after anatomic repair, 2 (22%) after aortic coarctectomy, and 2 (100%) after Norwood operation. Infants with a mitral valve diameter less than 2.5 x 10-2 m/m2 died at repair. In a mean follow-up of 73.5 months there were five secondary mitral valve plasties and three repairs after aortic coarctectomy. CONCLUSIONS: Among patients with partial atrioventricular canal, there is an important subgroup with clinical signs of heart failure in the first year of life. Left-sided obstructive lesions and complex mitral valve anomalies seem to play a fundamental role in the clinical evolution and prognosis of these patients. The echocardiographic mitral valve diameter may be useful for determining the correct surgical indication.

Right submammary minithoracotomy for repair of congenital heart defects.

OBJECTIVE: The initial experience with the right submammary minithoracotomy incision for correction of intracardiac congenital defects is reported. METHODS: Between March 1997 and March 1999, 100 children underwent repair of congenital heart disease through this approach. Their mean age and weight were 4.6 years and 20 kg, respectively. Diagnosis included: atrial septal defect (78), ventricular septal defect (7), tetralogy of Fallot (6), partial atrioventricular canal (5), double-chambered right ventricle (3) and single ventricle with dextrocardia (1). The standard technique entailed a 5 to 6 cm right submammary incision, entering the chest through the third or fourth intercostal space (depending on the body weight), direct aortic and bicaval cannulation and aortic cross-clamping with cardioplegic protection. RESULTS: There were no hospital deaths. Postoperative morbidity included bleeding in two cases, recurrent atrial septal defect in one, spleen injury in one. The average hospital stay was 3.5 days. All patient are currently free of symptoms and medications. CONCLUSIONS: (1) This approach for repair of selected congenital cardiac malformations is technically feasible, safe and effective; (2) younger age is a facilitating factor; (3) hospital stays are effectively reduced.