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B Marino

Publications and source records attributed to B Marino.

At least 19 recordsLinked to original sources
PubMed2002

Factors influencing immediate and long-term results after button's technique.

BACKGROUND: Aim of this study was to evaluate the factors influencing immediate and long-term results in patients undergoing aortic root replacement with a composite graft. METHODS: Between January 1989 and February 1999, 105 patients (83 males, 22 females) who underwent Bentall technique were studied. Preoperative diagnosis was annulo-aortic ectasia in 54, aortic dissection in 27, atherosclerotic aneurysm in 21, and aortitis in 3 cases. Seventeen patients were affected by Marfan's syndrome. All cases, elective, urgent, and emergency were included. Button technique was performed and the associated surgical procedures were coronary artery bypass grafting in 21, total aortic arch replacement in 15, proximal hemi arch in 5, and mitral valve replacement in 5 cases. RESULTS: The overall hospital mortality rate was 7.6% (n=8). Univariate analysis using chi(2) and/or two-sample "t"-test showed that dissection, aortitis, aneurysm rupture into-pleura or pericardium, emergency status, redo, prolonged pump times and circulatory arrest, were predictors influencing in-hospital mortality. Coagulopathy, low cardiac output, stroke, perioperative myocardial infarction, surgical bleeding leading to reoperation, were significantly related to in-hospital mortality (by correlation analysis). A multivariate analysis showed that, emergency status (p=0.027), aortic dissection (p=0.029), perioperative myocardial infarction (p=0.0021), reoperation for bleeding (p=0.0023), and pump time >180 min (p=0.011), were significant. The actuarial survival rate at 10 years follow-up was 84.7%. There were 8 late deaths. The Kaplan-Meier showed significant differences when considering dissection vs non-dissection (p=0.018), but did not reach significance in Marfan vs non-Marfan groups (p=0.83). NYHA class IV (p=0.052), previous cardiac surgery procedure (p=0.041), concomitant CABG (p=0.021), total aortic arch reconstruction (p=0.001), and mitral valve replacement (p=0.016), were identified as significant by Log Rank test. CONCLUSIONS: The Bentall procedure for aortic root replacement is safe and durable; in hospital mortality in elective status it was 1.28%; early and long-term mortality higher in patients with acute dissection. Six late deaths were procedures related. Sixty-six patients (76.4%) were in NYHA I class at follow-up. The incidence of late outcomes, thromboembolism (1.03%), graft infection (2.06%), pseudoaneurysm (0%), reoperation in ascending aorta or aortic valve (3.1%), operations on the remaining aorta (6.7%), and hemorrhage due to anticoagulant therapy (1.03%), are very low.

Actuarial Analysis↗
PubMed2001

Complete transposition of the great arteries: patterns of congenital heart disease in familial precurrence.

BACKGROUND: Transposition of the great arteries (TGA) is considered to be associated only rarely with genetic syndromes and to have a low risk of precurrence among relatives of affected patients. Because most family studies have involved a relatively small number of patients and evaluated all types of TGA as a single group, we performed a large, prospective study investigating the precurrence of congenital heart disease in families of children with complete, nonsyndromic TGA. METHODS AND RESULTS: From January 1997 through December 2000, 370 patients with nonsyndromic, complete TGA were consecutively evaluated and enrolled in the study. The occurrence of cardiac and noncardiac anomalies among relatives of the probands was investigated. Relatives with congenital heart disease were found in 37 of 370 families (10%), including 5 of 37 families (13.5%) with more than one affected relative. TGA itself was the most common precurrent malformation: complete TGA occurred in 6 families and congenitally corrected TGA occurred in 5 families. Precurrence risks for congenital heart disease were calculated at 1.8% (8 of 436) for siblings, 0.5% (4 of 740) for parents, 0.5% (16 of 3261) for first cousins, 0.2% (4 of 2101) for uncles/aunts, and 0.06% (1 of 1480) for grandparents. CONCLUSIONS: The present study shows that TGA is not always sporadic in families. Precurrence of concordant cardiac defects within affected family members supports monogenic or oligogenic inheritance of TGA in certain kindreds. Moreover, the occurrence of complete TGA and congenitally corrected TGA among first-degree relatives in several different families strongly suggests an underlying pathogenetic link between these 2 malformations that has been previously unrecognized.

Adolescent↗
PubMed2001

Congenital heart defects in Kabuki syndrome.

Kabuki makeup (Niikawa-Kuroki) syndrome (KS) is characterized by distinct facial anomalies, mental retardation, congenital heart defect (CHD), and skeletal malformations. In the present study we analyze cardiac characteristics and differences in sex prevalence of specific CHDs in our series of patients with KS and review published reports from the literature. Between January 1992 and February 2000, 60 patients (37 males and 23 females) with KS underwent phenotypic and cardiac evaluations at our hospital. CHD was diagnosed in 35 (58%) of our patients. Aortic coarctation (COA) (23%), atrial septal defect (ASD) (20%), and ventricular septal defect (VSD) (17%) were the most frequent CHDs in our series and in previous reports from the literature. Male preponderance was noted in patients with COA. In conclusion, CHD is a cardinal feature of KS. There is an overlap between cardiac malformations of KS and those of Turner syndrome. Male preponderance in patients with KS and COA supports the hypothesis that genes located on the X chromosome may be involved in determining KS in some patients. The high prevalence of CHD prompts accurate re-examination of patients evaluated by pediatric cardiologists in order to identify mild and still unrecognized cases of KS.

Abnormalities, Multiple↗
PubMed2001

Emergency operation for acute type A aortic dissection: neurologic complications and early mortality.

BACKGROUND: Acute type A aortic dissection is a surgical emergency still associated with high postoperative complications. The aim of this study was to investigate factors for hospital mortality and neurologic deficit in patients undergoing emergency operation for acute type A aortic dissection. METHODS: Eighty-five consecutive patients (age range, 20 to 82 years) operated on for acute type A aortic dissection over a 6-year period were evaluated. Univariate and stepwise multiple logistic regression analyses were conducted among 32 perioperative variables. RESULTS: All patients underwent surgical procedures under deep hypothermic circulatory arrest. Antegrade or retrograde cerebral perfusion was used in 23 patients (27.1%) and 18 patients (21.2%), respectively. Forty-three patients underwent arch/hemiarch replacement and the ascending aorta was replaced in 42 patients. Overall mortality rate was 25.9% (22 of 85 patients). Multiple logistic regression analysis showed that lack of cerebral perfusion (p = 0.021) and postoperative renal failure (p = 0.006) were the best predictors for hospital death. Twenty-one patients (24.7%) experienced neurologic accidents. The risk factor for postoperative neurologic complication was lack of cerebral perfusion (p = 0.013). Hospital mortality was 13% (3 of 23 patients) and 16.7% (3 of 18 patients) in the antegrade and retrograde cerebral perfusion groups (p > 0.05) and neurologic deficit was 13% (3 of 23 patients) and 11.1% (2 of 18 patients), respectively (p > 0.05). CONCLUSIONS: Hospital mortality and neurologic complications in patients undergoing emergent operation for acute type A aortic dissection were reduced when cerebral perfusion was used with deep hypothermic circulatory arrest.

Adult↗
PubMed2001

Arrhythmogenic right ventricular cardiomyopathy: clinicopathologic correlation based on a revised definition of pathologic patterns.

Different morphologic features of arrhythmogenic right ventricular cardiomyopathy (ARVC) have been described. However, it is still unclear whether they correspond to distinct forms of the same disease. A pathologic study was performed on a series of ARVC (15 from heart transplant and 12 from autopsy) from 2 Italian referral university hospitals. Based on both myocellular features and the nature of myocardial replacement, hearts were divided into 2 groups: infiltrative, with a lacelike pattern of transmural fatty infiltration and strands of normal residual cardiomyocytes (n = 11); and cardiomyopathic, with massive myocardial replacement by fibro fatty tissue and cardiomyopathic changes (such as hypertrophy and myofibril loss) of residual cardiomyocytes (n = 16). Hearts from the infiltrative group were mostly obtained at autopsy of patients who died suddenly. Fatty substitution was limited almost exclusively to the right ventricle. Mitral valve dysplasia (prolapse or cleft) was frequently present. Hearts from the cardiomyopathic group came mainly from heart transplants for congestive heart failure. Fibro fatty replacement was more extensive, usually biventricular. Active myocarditis and features suggestive of myocardial transdifferentiation were also observed. Despite these differences in clinical outcome and morphologic features, patients from the 2 groups showed similar mean age, sex distribution, occurrence of threatening ventricular arrhythmias, and prevalence of family history of sudden death, arrhythmias, or cardiomyopathy. Infiltrative and cardiomyopathic patterns represent different clinical and pathologic subsets of ARVC. Myocellular features are an important clue in the distinction between the two entities. The differentiation between the 2 patterns is feasible on endomyocardial biopsy and could give important prognostic information.

Adipose Tissue↗
PubMed2001

Modified autonomic balance in offsprings of diabetics detected by spectral analysis of heart rate variability.

This study was performed to evaluate the influence of family history for non-insulin-dependent diabetes mellitus (NIDDM) on autonomic balance. The latter was assessed by spectral analysis of heart rate variability (SA-HRV) and by analyzing the relative contribution of low-frequency (LF) and high-frequency (HF) components. Twenty glucose normotolerant offsprings of NIDDM parents and 20 controls underwent a 1-hour continuous electrocardiogram (ECG). LF and HF (mean +/- SEM in normalized units [NU]), respectively increased and decreased in offspring versus controls. The LF/HF ratio (mean +/- SEM) significantly increased (LF/HF = 3.25 +/- 0.7 v 1.45 +/- 0.5, P <.0001 offsprings v controls). To test a stimulated response, a passive tilting (+ 90 degrees ) after 30 minutes of bed rest (0 degrees ) was performed in a subsample of subjects (10 offsprings v 10 controls). During bed rest, we found significantly higher values of the LF/HF ratio in offsprings versus controls (1.93 +/- 0.3 v 1.08 +/- 0.2, P <.05), whereas in the head-up position, the LF/HF ratio value increased to the same levels in the 2 groups (6.48 +/- 1.3 v 6.89 +/- 1.4, not significant [NS]). NIDDM family history is characterized in the basal condition by an imbalance of the autonomic system, which, compared with controls, is expressed by a higher weight of sympathetic and a lower weight of parasympathetic components. No significant differences can be found under stimulated conditions.

Adult↗
PubMed2001

Clinical evaluation of St Jude Medical Hemodynamic Plus versus standard aortic valve prostheses: The Italian multicenter, prospective, randomized study.

OBJECTIVE: Hemodynamic and clinical performances of 21-mm and 23-mm St Jude Medical Hemodynamic Plus aortic valves (St Jude Medical, Inc, St Paul, Minn) were compared with those of 21-mm and 23-mm St Jude Medical standard cuff aortic valves in the first such multicenter, prospective, randomized study. Hemodynamic Plus valves are mechanical, bileaflet prostheses suitable for the small aortic anulus. METHODS: Patients with 21-mm and 23-mm anulus diameters were randomized to receive either a Hemodynamic Plus or a standard cuff valve. Postoperatively and at 6 months after the operation, patients underwent 2-dimensional Doppler echocardiography. Ejection fraction, cardiac output, peak gradient, mean gradient, effective orifice area, effective area index, and performance index were calculated. Postoperative and 6-month echocardiographic measurements and their variations across observation times were analyzed statistically. RESULTS: Of the 140 patients enrolled, 5 died at operation and 1 died of aortic dissection during the follow-up period. Eight patients were lost to follow-up. A total of 125 patients completed the study. In 1 patient a sewing cuff escaped intraoperatively. At 6 months the 21-mm and 23-mm Hemodynamic Plus valves showed significantly lower peak gradients and mean gradients than those of the 21-mm and 23-mm standard cuff valves. The 21-mm Hemodynamic Plus valves had gradients similar to those of the 23-mm Hemodynamic Plus valves. The effective orifice area did not differ significantly between the Hemodynamic Plus and standard cuff valves at either measurement. No valve mismatch was found in the 4 groups of patients. A more enhanced decrease of peak gradients and mean gradients and a more enhanced increase of effective orifice areas, effective area indices, and performance indices were found across observation times for patients with Hemodynamic Plus valves compared with those with standard cuff valves. CONCLUSIONS: Clinical hemodynamic performances of 21-mm and 23-mm St Jude Medical Hemodynamic Plus valves correspond closely with those of standard cuff valves, and gradients are substantially better than those of standard cuff valves of the same diameter. Therefore, use of this valve may minimize the need for aortic anulus enlargement. Early follow-up results with the Hemodynamic Plus valves were excellent, although more time is required to confirm this outcome.

Aortic Valve↗
PubMed2001

Aortopulmonary window coexisting with tetralogy of Fallot.

Aortopulmonary window is a rare cardiac malformation presenting either in the isolated form, or in association with other cardiac anomalies. The isolated form usually presents an increased pulmonary blood flow as the main clinical feature, but if associated with other cardiac anomalies the clinical findings change according to the additional defect. Only 19 cases of aortopulmonary window associated with tetralogy of Fallot have been reported. Five of them have associated pulmonary atresia. We report two neonatal cases of aortopulmonary window and tetralogy of Fallot, one of them presenting an associated pulmonary atresia.

Aortopulmonary Septal Defect↗
PubMed2001

Left ventricular thrombosis after blunt chest trauma.

A 22-year-old man was admitted to our observation with left ventricular thrombus arising after blunt chest trauma occurring during a ski accident one year before. None was obtained from a review of instrumental and laboratory data at trauma time. Transesophageal echocardiography showed an intraventricular thrombus and severe hypokinesia at the apex. Standard cardiac surgery procedure was performed and postoperative period was uneventful. Echocardiography controls at 6/12 months showed a normal apex kinesia. This case shows the importance of hospitalization, hemodynamics monitorization and late serial echocardiographic controls for timely diagnosis and management of myocardial contusion and consecutive ventricular thrombus formation to prevent life-threatening complications.

Adult↗
PubMed2001

Pulmonary atresia with ventricular septal defect: prevalence of deletion 22q11 in the different anatomic patterns.

BACKGROUND: Pulmonary atresia with ventricular septal defect (PA-VSD) is one of the most common cardiac defects associated with DiGeorge syndrome. The pattern of the pulmonary circulation determines the complexity of this type of heart disease. The aim of this study was to establish the prevalence of DiGeorge syndrome with deletion 22q11 in patients with simple and complex PA-VSD. METHODS: Since 1993 we have studied 128 consecutive patients affected by PA-VSD. In 90 of our patients the PA-VSD was considered "simple" (group I), because it was not associated with any other cardiac defects. In the other 38 children the PA-VSD was considered "complex" (group II) owing to the presence of heterotaxia, tricuspid atresia, a double-inlet left ventricle, transposition of the great arteries and congenitally corrected transposition of the great arteries. RESULTS: In group I, 38 patients (42%) had genetic syndromes or major extracardiac anomalies; deletion 22q11 was detected in 31% of cases. Major aortopulmonary collateral arteries were present in 50% of group I patients and in 57% of those with deletion 22q11. In group II, 10 patients (26%) had genetic syndromes or major extracardiac anomalies but none had deletion 22q11 (p < 0.005); in no case was the presence of major aortopulmonary collateral arteries observed (p < 0.005). CONCLUSIONS: PA-VSD is an anatomically and morphogenetically heterogeneous disease: in the setting of DiGeorge syndrome or velocardiofacial syndrome, PA-VSD is associated with a peculiar cardiac pattern and is due to deletion 22, whereas in case of nonsyndromic PA-VSD or when this disease is associated with different syndromes or with other types of cardiac defects, it is due to other morphogenetic mechanisms.

Child Welfare↗
PubMed2000

Heterotaxy with left atrial isomerism in a patient with deletion 18p.

We report on a female infant with partial deletion of the short arm of chromosome 18 (del 18p) and heterotaxy with left atrial isomerism. Congenital heart defect (CHD) is found in 10% of the literature reports. Interestingly, situs abnormalities have been diagnosed in four patients with del 18p, including ours. This finding could imply that a locus or loci involved in the development of normal body situs lies within this chromosomal region. Del 18p must be consid- ered when evaluating a patient with phenotypic anomalies and CHD in lateralization defects.

Chromosome Deletion↗
PubMed2000

Deletion of a 5-cM region at chromosome 8p23 is associated with a spectrum of congenital heart defects.

BACKGROUND: Cytogenetic evidence suggests that the haploinsufficiency of > or =1 gene located in 8p23 behaves as a dominant mutation, impairing heart differentiation and leading to a wide spectrum of congenital heart defects (CHDs), including conotruncal lesions, atrial septal defects, atrioventricular canal defects, and pulmonary valve stenosis. An 8p heart-defect-critical region was delineated, and the zinc finger transcription factor GATA4 was considered a likely candidate for these defects. We narrowed this region and excluded a major role of GATA4 in these CHDs. METHODS AND RESULTS: We studied 12 patients (7 had CHD and 5 did not) with distal 8p deletions from 9 families by defining their chromosome rearrangements at the molecular level by fluorescent in situ hybridization and short-tandem repeat analysis. Subjects with 8p deletions distal to D8S1706, at approximately 10 cM from the 8p telomere, did not have CHD, whereas subjects with a deletion that included the more proximal region suffered from the spectrum of heart defects reported in patients with 8p distal deletions. The 5-cM critical region is flanked distally by D8S1706 and WI-8327, both at approximately 10 cM, and proximally by D8S1825, at 15 cM. Neither GATA4 nor angiopoietin-2 (ANGPT2; a gene in 8p23 involved in blood vessel formation) were found to be deleted in some of the critical patients. We also found that CHDs are not related to the parental origin of deletion. CONCLUSIONS: Haploinsufficiency for a gene between WI-8327 and D8S1825 is critical for heart development. A causal relationship does not seem to exist between GATA4 and ANGPT2 haploinsufficiency and CHDs.

Adolescent↗
PubMed2000

Clinical and cardiorespiratory assessment in children with Down syndrome without congenital heart disease.

OBJECTIVE: To assess the clinical and functional status of a young Down syndrome (DS) population without congenital heart disease. DESIGN: Prospective study of children with DS and control subjects. SETTING: Bambino Gesù Children's Hospital, Rome, Italy. PARTICIPANTS AND METHODS: Forty-two children with DS (mean +/- SD age, 9.8 +/- 3.6 years) underwent genetic, clinical, neuropsychological (IQ), and cardiorespiratory evaluation. Cardiorespiratory fitness was assessed with a treadmill test and a lung function test to determine forced vital capacity, first-second forced expiratory volume, and peak expiratory flow. Data were expressed as a percentage of the predicted values for control children. To assess cooperation during exercise testing, we devised a compliance scale assigning a score according to the subjects' ability to understand instructions, ability to walk and run as required, and need for vocal encouragement. RESULTS: Eighteen (43%) of 42 children with DS were obese, 10 (24%) were short, and 17 (40%) had microcephaly. On the Leiter International Performance Scale, 2 of 35 subjects had a normal IQ score (80-120); all others had low IQ scores (79 to <40). Five subjects did not undergo cardiorespiratory assessment. Eighteen of 37 subjects completed lung function tests: the results for 10 were unremarkable, and results from 8 revealed reduced forced vital capacity because of poor compliance. The subjects tested had low exercise tolerance (mean +/- SD tolerance percentage, 61% +/- 12%), mild tachycardia (maximal heart rate, 91% +/- 4%), and a mild hypertensive response (maximal blood pressure, 89% +/- 8%). Compliance scores correlated significantly with exercise time and age but not with IQ. CONCLUSIONS: Clinical and cardiorespiratory assessment is feasible in subjects with DS without congenital heart disease and should be useful in gauging their fitness level for safe physical activity.

Adolescent↗
PubMed2000

Familial recurrence of nonsyndromic interrupted aortic arch and truncus arteriosus with atrioventricular canal.

BACKGROUND: Multifactorial inheritance is probably involved in most cases of nonsyndromic conotruncal heart defects (CHDs), but Mendelian transmission is often suspected. RESULTS: We report on a family with recurrence of nonsyndromic CTHD in two double first cousins; i.e., two brothers married two sisters. One of the cousins (case 1) had interrupted aortic arch (IAA) type B, while the other one (case 2) had truncus arteriosus (TA) with atrioventricular canal defect (AVCD). CONCLUSIONS: Our family further supports monogenic inheritance of CTHDs. In addition, the presence of TA associated with AVCD in one of the patients confirms the higher occurrence of CTHD in families with complex TA. The absence of 22q11 microdeletion (del22q11) in our patients, as in several literature reports of familial CTHDs, supports the existence of genes different to those located on chromosome 22q11 which could be implicated in the pathogenesis of CTHDs.

Aorta, Thoracic↗
PubMed2000

Adenosine triphosphate-dependent potassium channel modulation and cardioplegia-induced protection of human atrial muscle in an in vitro model of myocardial stunning.

OBJECTIVES: Although adenosine triphosphate-dependent potassium channel openers have been shown to enhance cardioplegic protection in animal myocardium, there is a lack of data on human cardiac tissues. We aimed at determining, on human atrial muscle, whether adenosine triphosphate- dependent potassium channels are involved in protection caused by high-potassium cardioplegia and whether adenosine triphosphate-dependent potassium channel activation might improve cardioplegic protection in an in vitro model of myocardial stunning. METHODS: Human atrial trabeculae were obtained from adult patients undergoing cardiac operations. In an organ bath at 37 degrees C, the preparations were subjected to 60 minutes of hypoxia at a high stimulation rate either in Tyrode solution (control, n = 17) or in St Thomas' Hospital solution without additives (n = 6) or associated with 100 nmol/L bimakalim (n = 7) or 1 micromol/L glibenclamide (n = 7), followed by 60 minutes of reoxygenation and 15 minutes of positive inotropic stimulation with 1 micromol/L dobutamine. RESULTS: Atrial developed tension was reduced by hypoxia to 27% +/- 5% of baseline and incompletely recovered after reoxygenation to 38% +/- 7%, whereas dobutamine restored contractility to 74% +/- 7% of basal values. St Thomas' Hospital solution with or without bimakalim improved developed tension after reoxygenation and dobutamine (P <.0001 vs control), whereas glibenclamide inhibited these protective effects of cardioplegic arrest (P =.001 vs St Thomas' Hospital solution). After reoxygenation, the protective effect of bimakalim disappeared at a high pacing rate (400- and 300-ms cycle length) but recovered during dobutamine superfusion. CONCLUSIONS: Adenosine triphosphate-dependent potassium channels are likely involved in the cardioprotective effects of cardioplegia in human atrial trabeculae and adenosine triphosphate-dependent potassium channel activation with bimakalim used as an additive to cardioplegia enhanced protection.

Adenosine Triphosphate↗