Subtotal anomalous pulmonary venous connection and left cor triatriatum: a rare diagnosis in adulthood.
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Adult↗
Biomedical subjects
Berardo Sarubbi
Publications and source records attributed to Berardo Sarubbi.
The Wolff-Parkinson-White electrocardiogram pattern in athletes: how and when to evaluate the risk for dangerous arrhythmias. The opinion of the paediatric cardiologist.
Although diagnostic assessment and treatment have been described in detail in patients with symptomatic Wolff-Parkinson-White (WPW) syndrome, the management of asymptomatic subjects remains controversial. Usually they are assumed to have a benign prognosis, although they do very occasionally present with ventricular fibrillation (VF) as the first manifestation of the syndrome. Discovering a WPW pattern in a previously asymptomatic athlete on a routine electrocardiogram (ECG) identifies the necessity for more accurate screening tests. However, non-invasive methods (Holter monitoring, exercise treadmill testing) seem to be relatively incomplete for risk stratification, especially for athletes. Current guidelines do not always recommend a routine electrophysiological study (EPS) in patients with an asymptomatic WPW ECG pattern, especially in children younger than 12 years. Individuals who engage in high-risk occupations or those patients who have a pre-excitation pattern which precludes them from following their chosen career or activities may be exceptions. The presence of inducible reciprocating tachycardia during EPS, especially when it triggers atrial fibrillation with short RR interval, can represent a specific risk marker of dangerous arrhythmias.
Child↗
Late cure after radiofrequency catheter ablation in a pediatric patient.
A 12-year-old female with the Wolff-Parkinson-White syndrome underwent an electrophysiologic study followed by radiofrequency catheter ablation of the left-lateral accessory pathway. After several unsuccessful attempts, the procedure was stopped because of early recurrence of accessory pathway conduction. Twenty-four hours after the procedure, the patient was found without ventricular pre-excitation pattern on the electrocardiogram. During a 12-month follow-up, the Wolff-Parkinson-White pattern was no longer present. A transesophageal electrophysiologic study showed no further tachycardia induction and the patient is still asymptomatic. This report raises the issue that ablation-induced lesions may evolve considerably during the first few days after ablation, leading to either recurrent accessory pathway conduction or long-lasting complete cure.
Catheter Ablation↗
A multicentre approach for the management of adults with congenital heart disease.
OBJECTIVE: At present, the level of care for adult congenital heart disease is not completely satisfactory in many European countries including Italy. The aim of this multicentre study was to evaluate the possibility of creating an active connection among different centres involved in the care of this patient population. METHODS: This study lasted two years, from December 2002 to December 2004. It involved six clinical centres located in the north, centre and south of Italy; a research centre (ISBEM) affiliated to the National Research Centre was also involved. Each centre was supposed to contribute with written information (about their specific subject) on the web site; our target readers were physicians, nurses and patients. The intranet part of the web connection was used for a registry to be filled with very simple information (demographic data, main diagnosis, reason for hospitalisation, type of treatment) about patients hospitalised after January 2000. The aim of the registry was to get a snapshot of the composition of the treated population, and the spectrum of the lesions. RESULTS: The main results are the following: (i) the creation of the web site www.guch-italia.it which can be accessed without a specific password; (ii) the creation of a registry in which we entered the clinical information of 1,231 patients; (iii) the presentation of guidelines on the web site, extrapolated from the recommendations for the management of adults with congenital heart disease from the Canadian Cardiovascular Society Consensus Conference. CONCLUSIONS: With this study we tried to create a national network of centres that have as an objective to optimise the assistance to patients with adult congenital heart disease, trying to make the approach as much homogeneous as possible.
Adolescent↗
Noninvasive risk stratification prevents sudden death due to paroxysmal atrial fibrillation in hypertrophic cardiomyopathy.
Sudden death is a tragic complication of hypertrophic cardiomyopathy. We report the case of a young patient with hypertrophic cardiomyopathy in whom an episode of atrial fibrillation triggered ventricular fibrillation and cardiac arrest. A 21-year-old man with nonobstructive hypertrophic cardiomyopathy underwent cardioverter-defibrillator implantation for primary prevention of sudden death, after risk stratification with noninvasive strategies. After 6 weeks, during a moderate effort, the patient had a syncopal episode, preceded by palpitations and dizziness, and terminated by the cardioverter-defibrillator. Device interrogation revealed an episode of atrial fibrillation with high ventricular response, spontaneously followed by ventricular tachycardia/fibrillation. Atrial fibrillation is a potential trigger of life-threatening arrhythmias and sudden death in patients with hypertrophic cardiomyopathy. Clinical investigation of risk markers for sudden death should be encouraged to identify high-risk patients who may benefit from a prophylactic therapy with an implantable cardioverter-defibrillator.
Adult↗
Left ventricular remodelling in outflow tract obstructive lesions during fetal life.
OBJECTIVE: Left ventricular (LV) remodelling in prenatally diagnosed LV outflow tract obstructive lesions such as aortic stenosis and aortic coarctation is important for prenatal counselling and postnatal management. The purpose of this study was to document the spectrum and the progression of different LV remodelling patterns and to identify prenatal markers of hypoplastic left heart syndrome (HLHS). METHODS: We studied 29 fetuses with LV outflow tract obstruction: 13 with isolated aortic stenosis, 14 with isolated aortic coarctation and two with combined aortic stenosis and aortic coarctation. Echocardiographic evaluation was performed 4 and 8 weeks after the first observation and at birth. RESULTS: None of the fetuses had HLHS (LV end-diastolic diameter z score higher than -2) at first prenatal echocardiography (24.5 + or - 3.6 weeks). Fetuses were divided into two groups: group A (n = 25) with a LV end-diastolic volume at birth > 20 ml/m(2); group B (n = 4) with a LV end-diastolic volume at birth < 20 ml/m(2) (LV hypoplasia). At first echocardiographic evaluation, the two groups showed a significantly different aorta to pulmonary ratio (0.44 + or - 0.08 vs. 0.86 + or - 0.14; P < 0.001); other LV echocardiographic features were not significantly different. The growth of the mitral (0.10 + or - 0.02 vs. 0.43 + or - 0.28 mm/week; P < 0.02) and aortic annulus (0.08 + or - 0.01 vs. 0.26 + or - 0.14 mm/week; P < 0.05) was significantly slower in group B. CONCLUSIONS: Our data suggest that LV outflow tract obstruction can progressively evolve in HLHS during pregnancy. A smaller aorta to pulmonary ratio was the only significant difference at initial echocardiographic evaluation in the two groups. Moreover, serial echocardiographic examinations are necessary to recognize fetuses at risk for HLHS caused by a subnormal growth rate of the mitral and aortic annulus.
Cardiac Surgical Procedures↗
[Organization of care for adults with congenital heart disease].
The increasing number of adult patients with congenital heart disease and the better survival of patients with complex disease into adulthood, as a result of the success of pediatric cardiology and cardiac surgery over the last years, have increased the need for specific structures, the so-called grown-up congenital heart disease units, able to provide comprehensive care to these patients. Many of the adult patients with congenital heart disease will require, over time, further operations, urgent in-hospital admission for a wide range of complications such as arrhythmias, hemorrhage, heart failure and bacterial endocarditis. Furthermore, these patients may often experience despair due to their awareness of residual morbidities and the knowledge of possible early mortality, or limitations in their social lives and educational or occupational attainment. Provision of care for children with congenital heart disease is well established in most parts of the world. In contrast, clinical services for the adults with congenital heart disease are scarce. In this scenario, adult cardiologists are not always equipped to deal with the range ad complexity of grown-up patients with congenital heart disease, whereas pediatric cardiologists cannot be expected to manage the many acquired adult diseases in a pediatric medical environment.
Adolescent↗
Left ventricular hypertrophy in Caucasian master athletes: Differences with hypertension and hypertrophic cardiomyopathy.
AIM: To study, by conventional echocardiography, left ventricular remodelling and function in master athletes, hypertension and hypertrophic cardiomyopathy. METHODS: We studied 30 master athletes (MA; soccer players; mean age 43.9+/-5.9), 24 subjects with essential hypertension (HYP; 46.6+/-6), 20 patients with hypertrophic cardiomyopathy (HCM; 42.2+/-9) and 30 normal individuals (CG; 43.4+/-5). An integrated M-mode/two-dimensional echocardiographic analysis was performed to determine chambers dimensions, relative wall thickness (RWT) and left ventricular mass (LVM), indexed to height in meters raised to the power of 2.7 (LVM/h(2.7)). Cut-off levels for LVM/h(2.7) and RWT were defined to assess 4 different patterns of LV geometric remodelling. In addition, we measured indexes of global systolic performance and indexes of global diastolic function. RESULTS: LV wall thickness and LV end-diastolic dimensions were higher in MA than controls, but significantly lower than other groups. LVH/h(2.7) was increased in 79% of HYP and in 95% of HCM, but was within the normal limits in MA. LV geometry was normal in 22 out of 30 MA (73%), while the remaining (8 athletes, 27%) showed a concentric remodelling. Systolic function (FS and EF) was normal in MA, but was slightly reduced in HYP and increased in HCM. Analysis of diastolic function showed an abnormal relaxation pattern in all HYP and 95% of HCM, but was normal in all MA. The ratio between peak filling rate and stroke volume (PFR/SV), a relatively independent index of diastolic function, was significantly greater in hypertensive patients with normal LV remodelling compared to those without it (4+/-0.39 vs. 4.91+/-0.19; P = 0.0002). CONCLUSION: MA showed lower values of wall thickness, LV dimensions and LV mass compared with HYP and HCM. Despite an abnormal remodelling, all the athletes showed a normal systolic and diastolic function. The differential diagnosis between MA, HYP and HCM is feasible by accurate, comprehensive standard Doppler echocardiography.
Adult↗
Congenital heart disease in a population of dizygotic twins: an echocardiographic study.
BACKGROUND: Congenital heart disease (CHD) is the most common malformation in the fetal and neonatal period but little is known about its cause. The distribution analysis of CHD in dizygotic twins could provide a useful tool to evaluate the role of genetic and environmental factors in the development of CHD. Dizygotic twins are siblings with different genes, growing together in the same womb. AIM OF STUDY: To investigate the occurrence of CHD in a large sample of dizygotic twins of nonconsanguineous healthy parents, comparing the data from non-twin patients. METHODS: From January 1999 to December 2002, we enrolled 1743 CHD patients with, at least 1 sibling, and 66 pairs of dizygotic twins, referred to our tertiary center. The diagnosis of CHD was based on clinical and echocardiographic evaluation. RESULTS: Considering only the sibling nearest in age for each non-twin patient the recurrence was 67/1743 (3.8%). Among these 67 patients, 35 (52.2%) had a sibling with the same or similar CHD. Conversely, considering all 1886 siblings, recurrence of CHD in the non-twin group was 70/1743 (4%). Of the 70 patients, 36 (36/70, 51.4%) had a sibling with the same suspected pathogenic mechanism of CHD. In 9/66 pairs of twins (13.6%), both siblings had a CHD. In the nine pairs of twins in whom both siblings had a CHD, the percentage of concordance (based on the suspected pathogenic mechanism) for CHD was 100% (p<0.05). CONCLUSIONS: Our findings suggest that the higher recurrence and concordance of CHD found in dizygotic twins could depend on some poorly identified environmental risk during the pregnancy.
Adult↗
Electrophysiological evaluation of asymptomatic ventricular pre-excitation in children and adolescents.
BACKGROUND: Diagnostic assessment and treatment have been described in detail in symptomatic WPW syndrome, but little information exists about significance and prognosis of an incidentally found ventricular pre-excitation (VPE) in asymptomatic children. The aim of the study was to evaluate, retrospectively, the role of electrophysiological study (EPS) in the assessment of the arrhythmic risk in asymptomatic patients with VPE. MATERIAL AND METHODS: Sixty-two asymptomatic children and adolescents (38 M/24 F, aged 9.8+/-5.1 years) referred to our Division between 1996 and 2002 for an incidentally found VPE underwent an EPS for arrhythmic risk stratification. The following parameters were examined: anterograde effective refractory period of the accessory pathway (AP), the 1-to-1 conduction over the AP, the inducibility of atrio-ventricular re-entrant tachycardia (AVRT) and the inducibility of atrial fibrillation (AF) with measurement of minimal RR between two consecutive preexcitated QRS complexes, the average RR interval of all cycles, and the percentage of preexcitated QRS complexes. RESULT: During the EPS, 36 patients (58.1%) experienced sustained SVT. The tachycardia was initiated in the basal state in 22 patients and after isoproterenol in the other 14. Orthodromic AVRT (cycle length 305.9+/-48.5 ms) was recorded in 29 patients. In three patients, both orthodromic and antidromic AVRT were recorded, with different cycle length (CL). Antidromic AVRT alone (CL 239.5+/-13.7 ms) was recorded in four patients. AF was recorded in nine patients: in six patients, it was recorded after the induction of orthodromic or antidromic AVRT, in the other three cases AF was the first and only arrhythmic event. The minimal RR between two consecutive pre-excitated QRS ranged between 250-230 ms (mean 237.5+/-9.6 ms). In the 26 patients who presented no induced sustained tachycardia in the EPS, the 1:1 conduction over the AP ranged between 210 and 600 ms (mean 279.6+/-75.2 ms). CONCLUSIONS: Electrophysiological evaluation remains the gold standard for assessing risk of life-threatening arrhythmias in patients with VPE. However, a high proportion of healthy children and adolescents with VPE can experience sustained AVRT and/or AF during EPS. These results raise questions about the necessity of an aggressive treatment approach to prevent those "rare" cases of sudden death.
Adolescent↗
Percutaneous treatment of moderate-to-large patent ductus arteriosus with different devices: early and mid-term results.
BACKGROUND: Transcatheter closure is now accepted as the first-choice therapeutic option in patients with patent ductus arteriosus (PDA). However, this procedure is still challenging in large PDA and/or younger patients. This study evaluated feasibility and results of this approach in large, symptomatic PDA using different devices. METHODS: Between April 2000 and July 2004, 57 patients underwent attempt of transcatheter closure of a large PDA at our Institution. Nineteen patients (33.3 %) were on pharmacologic therapy for congestive heart failure. PDA diameter was 3.2+/-1.2 mm (range 1.8-9 mm), resulting in a pulmonary to systemic flow ratio of 2.1+/-1.8 (range 1.4-5). RESULTS: The procedure was successfully performed in 54 patients (94.7%), using the Amplatzer duct occluder (ADO) device (34 patients) or a multiple detachable coil approach (20 patients). Complete PDA occlusion was recorded in 77.8% of patients at 24 hours, 92.6% at 1 month, and 94.4% at last follow-up control (23+/-12 months). PDA morphology and pulmonary to systemic flow ratio did not influence the success rate of the procedure or the residual shunt. A trend toward a higher occlusion rate at any follow-up point was recorded in the ADO group (79.5 vs 75.0% at 24 hours, 97.1 vs 85.0% at 1 month, and 97.1 vs 90% at last follow-up control, p = NS for all comparisons). CONCLUSIONS: Percutaneous closure might be considered effective and safe also in large, clinically significant PDA, by tailoring the device choice to the patient size and ductal morphology. In this setting, the multiple coil option revealed as effective as the ADO device over a mid-term follow-up.
Adolescent↗
Early electrical and geometric changes after percutaneous closure of large atrial septal defect.
Cardiac arrhythmias and right chamber enlargement are well known long-term sequelae of atrial septal defect (ASD). Surgical ASD closure relieves patient symptoms but often fails to revert cardiac volume overload findings. Transcatheter ASD closure might be an attractive alternative to surgery, also because of the possibility to study the amount and time-course of the electro-geometric modifications following shunt disappearance. Between March 2000 and December 2002, 24 patients (age 22.7 +16.8 years) underwent percutaneous closure of large ASD (stretched diameter >20 mm and/or QP/QS ratio >1.5:1). ASD closure was performed with the Amplatzer Septal Occluder device (mean 25 +/- 7 mm), achieving a complete occlusion in all patients at 1 month. In 6 patients, right ventricular (RV) monophasic action potential was recorded during the procedure. All patients underwent standard 12-lead electrocardiography and transthoracic echocardiography before and at 24 hours and 1 month after ASD closure. After the procedure, monophasic action potential length increased from 359 +/- 27 to 372 +/- 27 ms (p <0.0001). At 1 month, QT dispersion decreased from 54 +/- 25 to 41 +/- 17 ms (p <0.05), RV diastolic diameter decreased from 42 +/- 6 to 34 +/- 5 mm (p <0.00001), and left ventricular (LV) diastolic diameter increased from 39 +/- 5 to 44 +/- 5 mm (p <0.0001), resulting in a decrease in the RV/LV ratio from 1.11 +/- 0.22 to 0.79 +/- 0.11 (-28.8%, p <0.00001). Electrocardiographic changes, as well as the amount and time-course of RV overload relief, did not significantly differ between pediatric (<16 years of age; n = 11) and adult patients (n = 13). In conclusion, regardless of age at procedure, percutaneous ASD closure results in early striking electrical and geometric cardiac changes that may be beneficial during long-term follow-up.
Action Potentials↗
Electrophysiological changes following balloon valvuloplasty and angioplasty for aortic stenosis and coartaction of aorta: clinical evidence for mechano-electrical feedback in humans.
BACKGROUND: Basic research and animal experiments have shown electrophysiological changes during or after changes in mechanical loading. Electrical instability following mechanical stretch has been observed as development of after-depolarisation and dispersion of refractoriness and repolarisation. The aim of the present study was to evaluate the presence of the mechano-electrical feedback in humans, assessing the ventricular repolarisation changes following acute changes in left ventricular pressure. MATERIAL AND METHODS: The study group comprised 30 consecutive patients (22 M and 8 F, aged 2 days-24 years) affected by severe congenital aortic stenosis and 30 patients (20 M and 10 F, aged 6 months-16 years) affected by severe coartaction of aorta. Ventricular repolarisation was evaluated before and after percutaneous balloon valvuloplasty and angioplasty in terms of absolute measures (JT, JTc, QT, QTc) and in terms of dispersion across the myocardium: QT and QTc dispersion (QTD, QTcD), JT and JTc dispersion (JTD and JTcD) and T-peak to T-end interval (Tp-Te). RESULTS: Patients with severe aortic stenosis and patients with aortic coartaction showed a significant decrease in dispersion of ventricular repolarisation time indexes (QTD, QTcD, JTD, JTcD and Tp-Te) following valvuloplasty and angioplasty. CONCLUSIONS: Changes in hemodynamic loading can also produce electrophysiological effects in humans. Acute reduction in left ventricular pressure overload following balloon valvuloplasty and angioplasty, decreases electrical instability, as expressed by the reduction across the myocardium of the dispersion of ventricular repolarisation.
Adolescent↗
Isolated anomalous origin of the right pulmonary artery from the ascending aorta (so-called "hemitruncus"): diagnosis and complex management in a newborn.
We report a case of an anomalous origin of the right pulmonary artery (RPA) from the ascending aorta diagnosed at echocardiography at 13 days of age. The diagnostic clue was relieved in the suprasternal and parasternal high short-axis views, showing aorto-RPA continuity with a systolic flow in the left pulmonary artery and a systo-diastolic flow in the RPA. At 34 days of age the infant was submitted to surgery during which a direct end-to-lateral anastomosis without conduit interposition was performed. During the short-term follow-up the patient developed RPA stenosis at the anastomosis site and underwent percutaneous stent implantation.
Anastomosis, Surgical↗
A "multisite" atrioventricular block.
A 6-year-old child, operated for a perimembranous ventricular septal defect, underwent an electrophysiologic study for the presence of first degree atrioventricular (AV) block and bifascicular block with episodes of type 1 and type 2 second degree AV block. Electrophysiologic study showed a considerable infra-His conduction delay (HV interval 170 ms) and spontaneous phases of infra-His type 1 second degree AV block. During incremental atrial pacing supra-His type 1 second degree AV block and 2:1 infra-His AV block were simultaneously observed and this condition persisted unmodified despite the intravenous injection of atropine. The block distal to the His bundle was considered functional.
Child↗
Fetal supraventricular tachycardia diagnosed and treated at twenty-four weeks of gestation and after birth: a case report.
Supraventricular tachycardia is the most common clinically significant fetal tachycardia. The diagnosis is usually made at routine sonographic workup during the second-third trimester of pregnancy. Treatment goals are cardioversion to sinus rhythm and reversal of cardiac dysfunction. We describe a case of fetal supraventricular tachycardia diagnosed at 24 weeks of gestation. The first-line treatment was oral maternal digoxin and sotalol. This therapy was not sufficient for complete control of the tachycardia. Hence, second-line treatment with digoxin and flecainide was started and successfully achieved conversion to sinus rhythm. No adverse maternal side effects were noted during the 14 weeks of therapy. A normal male infant was delivered at elective cesarean section performed for obstetric indications at 38 weeks of gestation. A persistent junctional reciprocating tachycardia with a ventriculo-atrial/atrioventricular ratio > 1 was diagnosed following delivery at transesophageal electrophysiological study. At the age of 8 months the child is on therapy with sotalol (4 mg/kg/day) and flecainide (3 mg/kg/day) and is in good clinical conditions.
Adult↗
Intra-atrial re-entrant tachycardia with Wenckebach periodicity.
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Journal Article↗
Congenital junctional ectopic tachycardia: presentation and outcome.
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Journal Article↗