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Methods and results A 70-year-old woman presented to our outpatient clinic complaining of worsening dyspnoea in the last 3 months. She had a medical history of hypertension, diabetes, dyslipidemia, and paroxysmal atrial fibrillation. We performed a comprehensive evaluation starting with a transthoracic echocardiogram that showed a dilatation of right ventricle with normal function, severe pulmonary regurgitation, and moderate tricuspid regurgitation with estimated pulmonary artery systolic pressure of 55 mmHg; the left ventricle had normal dimension and function, with mild aortic and mitral regurgitation, and a subaortic membrane which caused a mild obstruction (maximum gradient 17 mmHg). The cardiac magnetic resonance (CMR) confirmed the enlargement of the right ventricle and of the pulmonary artery trunk (51 mm) and the severity of pulmonary regurgitation (regurgitant fraction of 41%). CMR also clearly showed the VSD just below the subaortic membrane and the left to right shunt with a jet that appeared to proceed straight from the left ventricle through the pulmonary valve (Figure 1A). The estimated Qp/Qs was 1.6 and no intramyocardial late enhancement was present. Pulmonary pressures and pulmonary vascular resistance were normal at the right heart catheterization and the Qp/Qs ratio calculated invasively was 1.45. Considering patient high-risk profile for coronary artery disease, a coronary angiography was also performed showing an abnormal origin of the right coronary artery (RCA) from the mid-portion of the left anterior descending coronary artery (LAD) with two significant stenosis: one involving the bifurcation of RCA and the other the mid-portion of the LAD (Figure 1B). The coronary computed tomography angiography (CCTA) showed a benign course of the RCA anterior to the pulmonary artery towards the auriculoventricular groove (Figure 1C, D). Taking into account all these findings, multidisciplinary heart team decided to perform a cardiac surgery intervention of pulmonary valve and trunk replacement, closure of ventricular septal defect and two coronary bypass grafts on LAD and RCA. Conclusions This case represents a combination of some rare congenital heart abnormalities where multimodality cardiovascular imaging techniques were essential to establish a proper diagnosis and to plan an adequate surgical repair. We hypothesize that the peculiar orientation of the VSD jet may have caused the pulmonary trunk dilatation considering that neither the shunt, nor the pulmonary pressure appear to have been of sufficient magnitude to cause it. Pulmonary ectasia and the damage inflicted by the jet to the cusps of the valve have led to the severe valvular insufficiency. While aortic and tricuspid regurgitation are known to be associated with VSD, to the best of our knowledge this is the first report of pulmonary regurgitation secondary to VSD.

Highlights • Sudden cardiac death (SCD), especially in young athletes, should be avoided. • Current pre-sports screening is based on history/physical exam (insufficient). • ECG is an excellent test for electrically induced SCD but requires expert review. • Screening MRI is the best tool for identifying myocardiopathies/coronary anomalies. • Results of a preliminary population-based study confirm the value of screening MRI., Preventing sudden cardiac death (SCD) in athletes is a primary duty of sports cardiologists. Current recommendations for detecting high-risk cardiovascular conditions (hr-CVCs) are history and physical examination (H&P)-based. We discuss the effectiveness of H&P-based screening versus more-modern and accurate methods. In this position paper, we review current authoritative statements and suggest a novel alternative: screening MRI (s-MRI), supported by evidence from a preliminary population-based study (completed in 2018), and a prospective, controlled study in military recruits (in development). We present: 1. Literature-Based Comparisons (for diagnosing hr-CVCs): Two recent studies using traditional methods to identify hr-CVCs in >3,000 young athletes are compared with our s-MRI-based study of 5,169 adolescents. 2. Critical Review of Previous Results: The reported incidence of SCD in athletes is presently based on retrospective, observational, and incomplete studies. H&P’s screening value seems minimal for structural heart disease, versus echocardiography (which improves diagnosis for high-risk cardiomyopathies) and s-MRI (which also identifies high-risk coronary artery anomalies). Electrocardiography is valuable in screening for potentially high-risk electrophysiological anomalies. 3. Proposed Project: We propose a prospective, controlled study (2 comparable large cohorts: one historical, one prospective) to compare: (1) diagnostic accuracy and resulting mortality-prevention performance of traditional screening methods versus questionnaire/electrocardiography/s-MRI, during 2-month periods of intense, structured exercise (in military recruits, in advanced state of preparation); (2) global costs and cost/efficiency between these two methods. This study should contribute significantly toward a comprehensive understanding of the incidence and causes of exercise-related mortality (including establishing a definition of hr-CVCs) while aiming to reduce mortality.

Objectives The purpose of this study is to compare the long-term outcomes of patent foramen ovale (PFO) closure using angiography or transesophageal echocardiography as procedural guidance. Background The interventional treatment is emerging as a safe and efficient option for patients with high likelihood of PFO-related cryptogenic stroke and high risk of recurrence. The "gold-standard" guidance technique remains an issue. Methods Two cohorts of patients undergoing PFO closure for cryptogenic stroke in two catheterization laboratories of the same institution, using similar inclusion criteria but different guidance, were compared with propensity score matching. Results A total of 374 patients were enrolled, 161 in Angio-group and 213 in Echo-group. No difference was detected in the procedural complication rate. In Angio-group, radiological exposure (P = .001) and 6-month residual shunt (16.8% vs 8.0%, P = .015) were higher. After a mean follow-up of 41 ± 30 months, 28 patients (7.5%) presented any adverse event (death, recurrent cerebral ischemia, device-related complications, reintervention), with a higher rate in Angio-group (13.0% vs 3.3%, P = .001), mainly due to repeated percutaneous intervention (10.6% vs 1.4%, P = .001). The results were confirmed after propensity score matching (118 patients/group). The rate of recurrent cerebral ischemia was 1.9% and was not significantly different in the two groups. Intra-procedural guidance and atrial septum aneurysm were independent predictors of the composite primary endpoint (OR 1.2, P = .016). Conclusions The use of intra-procedural transesophageal echocardiography (TEE) guidance for PFO closure allows lower residual shunt rate, radiological exposure, and adverse events, mainly driven by a significant reduction in percutaneous reintervention.

Cardiac involvement in familial amyloid polyneuropathy consists of arrhythmias, conduction disturbances, and heart failure. To our knowledge, heart rupture has never been described in association with this condition. We report the case of a 62-year-old man with a 6-year history of refractory familial amyloid polyneuropathy who underwent liver transplantation. The operation was complicated by severe hypotension because the neuropathy involved the autonomic system. Perioperatively, the patient had a myocardial infarction, and during the next 10 days, a complete interventricular septal rupture developed, resulting in a systemic-to-pulmonary shunt. Coronary angiographic findings were normal. However, the shunt caused unstable hemodynamics, resulting in cardiogenic shock. An attempt to close the rupture percutaneously failed. The patient underwent successful heart transplantation 50 days later. Macroscopic examination of the explanted heart showed thickening of both ventricles, septal rupture, and a gray scar in the interventricular septum around the cavity. Histopathologic examination revealed intramural amyloid angiopathy. Our case shows that heart rupture can occur in patients with familial amyloid polyneuropathy who have no history of obstructive coronary artery disease, perhaps as a result of tissue fragility caused by amyloid angiopathy. Therefore, autonomic disturbances should be regarded with concern and promptly treated in the perioperative period.

Transvenous lead extraction (TLE) of the Starfix coronary sinus (CS) active-fixation lead may be challenging, due to undeployment of fixation lobes and venous occlusion. We report our experience in Starfix TLE, in comparison with previous data. A 78-year-old male, implanted in 2009 with Starfix lead, was referred to our institution for TLE, due to infective endocarditis with lead-associated vegetations. The tip of Starfix lead was located in distant, anterior position, in the great cardiac vein, close to patent left internal mammary artery-to-left anterior descending artery anastomosis, and first-choice surgical removal had a prohibitive operative risk. Conventional dilatation beyond CS ostium, as well as the use of a standard delivery catheter, was ineffective. An off-label modification of the delivery, by cutting the distal soft tip, was successful. However, the tip of the lead fragmented and was trapped in the innominate vein. Then a gooseneck snare grasped the fragment, allowing complete retrieval. TLE of Starfix leads may be particularly challenging, especially when its tip is located in a distant anterior location. In these cases, important help may be obtained by dilatation within the CS, by means of conventional or modified delivery catheters. Only experienced operators, sometimes with non-conventional techniques, should perform TLE of Starfix leads. Learning objective: TLE of Starfix leads may be challenging, particularly when the tip is located in a distant anterior position. Dilatation with conventional tools may be precluded. In these cases modifications of the delivery catheters may be useful. Surgery should be avoided as first-choice procedure; only experienced operators, sometimes with non-conventional techniques, should perform TLE of Starfix leads.>

A ductus arteriosus aneurysm (DAA) was corrected with an 18 mm Amplatzer patent foramen ovale occluder. DAA is a rare finding, with sporadic cases reported (the vast majority in children and infants). In the elderly, it poses serious therapeutic challenges, as the risk of rupture is counterbalanced by the high risk of its correction, which requires surgery or placement of an endovascular prosthesis in a critical region such as the aortic arch.

Introduction The management of patients with residual right-to-left shunt (rRLS) after percutaneous patent foramen ovale (PFO) closure is debated. The aim of this study was to define the incidence of moderate-to-large rRLS and to report the feasibility, safety and long-term clinical outcome of transcatheter closure of rRLS. Methods and Results From June 2000 to March 2013, 322 subjects underwent percutaneous PFO closure. In 39 patients (12.1%) with moderate-to-large rRLS on transcranial Doppler (TCD) and/or transesophageal echocardiogram a second cardiac catheterization was performed with the aim of completing the closure. A second closure device was implanted in 21 patients (53.8%). In the remaining 18 (46.2%), a second device was not delivered for the following reasons: in 13 (72.2%) no residual passage could be crossed, in 5 (27.8%) the residual shunt was deemed to be negligible. No complications occurred. After the second procedure, complete closure was proved by TCD in 16/21 (76.2%) subjects. One patient received a third device. During follow-up (41 ± 19 months), no cerebrovascular ischemic accidents occurred. Conclusion A second percutaneous PFO occlusion device can be safely implanted in patients with significant rRLS. However, a moderate-to-large rRLS on TCD and/or TEE may not necessarily represent a significant risk of further paradoxical embolization.

ObjectivesTo retrospectively evaluate the impact on daily activities of transcatheter closure of patent foramen ovale (PFO) versus medical therapy in patients with migraine and to analyze the role of the residual shunt after PFO closure.BackgroundWhile non-controlled observational studies reported an improvement of migraine after PFO closure, a randomized trial has shown no benefit of such an intervention. The role of residual shunt after PFO closure is also poorly known.MethodsOut of 217 patients with migraine and echocardiographic evidence of PFO, 89 were managed with percutaneous PFO closure (Group A) while 128 were medically treated (Group B). All MIDAS questionnaires were obtained at the first evaluation and repeated at least 6 months after the index evaluation or after the PFO closure. All the patients were also asked to give a subjective estimate of their migraine status. A postprocedural transcranial Doppler study was available in 70 patients in Group A.ResultsThe mean basal MIDAS score did not differ between the two groups (p=0.859). After a mean follow-up (FU) of 1399±982 days the MIDAS score decreased significantly in both groups (Group A baseline vs FU, p

AIM There is still controversy regarding the benefit of percutaneous closure of patent foramen ovale (PFO) among patients with cryptogenic stroke. Here we aimed to evaluate the factors associated with treatment choice and predictors of adverse events in patients with cryptogenic stroke or transient ischemic attack (TIA) and PFO. METHODS Of 418 consecutive patients with PFO and cryptogenic stroke or TIA, 262 underwent percutaneous PFO closure, whereas 156 were medically treated. Multivariable logistic regression models were developed to evaluate factors influencing the treatment strategy and predictors of outcome, a composite of stroke, TIA or all-cause mortality. RESULTS Patients with large interatrial right-to-left shunt were more likely treated with percutaneous closure [odds ratio (OR) = 4.79, 95% confidence interval (2.73-8.42); P

For more than 70 years, early repolarization has been considered to be a common normal variant. In the general population, the prevalence ranges between 5 and 13%, and in athletes, a rising trend is observed from 20 to 90%. Nevertheless, from the latter half of the 1990s, a growing number of case reports, series, observational and prospective studies reported that the presence of various electrocardiographic patterns attributed to early repolarization may constitute a potential marker for the increased risk of sudden death in otherwise normal individuals, casting a dark shadow on this ECG peculiarity. This review provides a historical summary of the evolution of the concept of early repolarization from its original description to the latest works and a guide to help physicians in evaluating individuals with this common electrocardiographic pattern.

Infection of devices for percutaneous transcatheter closure of atrial septal defects are exceedingly rare. Two cases of device-associated endocarditis have been reported, which were both operated on. We describe the successful treatment with antibiotics of a device-associated endocarditis.

OBJECTIVES We aimed to evaluate: 1) the behavior of electrical activity simultaneously in different atrial regions during atrial fibrillation (AF); 2) the difference of atrial activation between paroxysmal and chronic AF; 3) the atrial refractoriness dispersion; and 4) the correlation between the effective refractory periods (ERPs) and the FF intervals. BACKGROUND Little data exist on the electrophysiologic characteristics of the different atrial regions in patients with AF. A more detailed knowledge of the electrical activity during AF may provide further insights to improve treatment of AF. METHODS Right and left atria were extensively mapped in 30 patients with idiopathic AF (18 paroxysmal and 12 chronic). In different atrial locations, we analyzed 1) the FF interval duration; and 2) the grade of organization and, in case of organized electrical activity, the direction of atrial activation. Furthermore, in patients with paroxysmal AF, we determined the atrial ERP, evaluated the ERP dispersion and assessed the presence of a correlation between the ERPs and the FF intervals. RESULTS In patients with chronic AF, we observed a shortening of the FF intervals and a greater prevalence of disorganized activity in all the atrial sites examined. In patients with paroxysmal AF, a significant dispersion of refractoriness was observed. The right lateral wall showed longer FF intervals and more organized atrial activity and, unexpectedly, the shortest mean ERPs. In contrast, the septal area showed shorter FF intervals, greater disorganization and the longest mean ERPs. CONCLUSIONS Electrical activity during AF showed a significant spatial inhomogeneity, which was more evident in patients with paroxysmal AF. The mean FF intervals did not correlate with the mean ERPs.

The management of patients with residual right-to-left shunt (rRLS) after percutaneous patent foramen ovale (PFO) closure is debated. The aim of this study was to define the incidence of moderate-to-large rRLS and to report the feasibility, safety and long-term clinical outcome of transcatheter closure of rRLS.From June 2000 to March 2013, 322 subjects underwent percutaneous PFO closure. In 39 patients (12.1%) with moderate-to-large rRLS on transcranial Doppler (TCD) and/or transesophageal echocardiogram a second cardiac catheterization was performed with the aim of completing the closure. A second closure device was implanted in 21 patients (53.8%). In the remaining 18 (46.2%), a second device was not delivered for the following reasons: in 13 (72.2%) no residual passage could be crossed, in 5 (27.8%) the residual shunt was deemed to be negligible. No complications occurred. After the second procedure, complete closure was proved by TCD in 16/21 (76.2%) subjects. One patient received a third device. During follow-up (41 ± 19 months), no cerebrovascular ischemic accidents occurred.A second percutaneous PFO occlusion device can be safely implanted in patients with significant rRLS. However, a moderate-to-large rRLS on TCD and/or TEE may not necessarily represent a significant risk of further paradoxical embolization.

The case of a 60 year-old woman who underwent successful tricuspid valve-in-valve implantation for an early degenerated bioprosthesis was previously described [1]. After 14 months of well-being the patient experienced once again exertional dyspnoea. Clinical examination was unremarkable: there were neither heart murmurs nor peripheral oedema. A cardiopulmonary test showed mild depression of functional capacity (VO2 peak of 19.7 ml/kg/min, 85% of the theoretical expected value), and moderate desaturation (SO2 88% after four minutes). At the transthoracic echocardiogram the Sapien XT prosthesis in tricuspid position was found to have a mean gradient of 4 mmHg, and minimal, physiological, central regurgitation (Fig. 1A). A bubble-contrast showed a rightto-left shunt at rest, through a patent foramen ovale (PFO)

Although atherosclerotic disease often becomes clinically evident only in one particular vascular distribution during a person's lifespan, it is an ubiqui- tous process, that can be detected in all arteries, depending on the diagnostic test adopted and the threshold chosen. Disease of the carotid arteries is frequent and the present chapter will focus on the correlation of this pathology with coronary heart disease.

A 41 -year-old woman was referred to our Cardiology Unit to evaluate the feasibility of a percutaneous closure of her 20-mm atrial septal defect. During the last few months she had increasing dyspnoea, and an echocardiographic study had disclosed an atrial septal defect with significant right ventricular enlargement. Her past medical history was silent, except for Nickel (Ni 2+ ) allergy confirmed by a skin patch test. Ni 2+ allergy is a problematic issue in patients scheduled for Ni 2+ -containing devices. The atrial septal occluder device that we used consists of a metallic alloy composed of 55% nickel and 45% titanium. Starting from the aforementioned case we reviewed all available literature and illustrated our final resolution.

The correlation analysis showed a good overlap between measurements (Qp: r = 0.65, p = 0.0004; Qs: r = 0.64, p = 0.0005; Qp/Qs ratio: r = 0.68, p = 0.0002), also confirmed by regression analysis (R2 = 0.42, p < 0.001 for Qp; R2 = 0.41, p = 0.001 for QS; R2 = 0.46, p < 0.001 for Qp/Qs ratio), and by the Bland-Altman statistical analysis for method comparison (see Figure 1). The interobserver variability was low.

Non-cardiac chest pain is a frequent finding in patients admitted to emergency departments, and it has been shown that many of these patients may have an esophageal cause for their pain. However, little data are available on patients primarily referred to the cardiology unit, and especially those with coronary artery disease. The purpose of this study was to assess the role of esophageal dysfunction in chest pain patients with and without coronary artery disease.Eighty-one patients referred from a cardiology unit for chest pain and no myocardial infarction entered the study. Sixty-one patients had no evidence of coronary artery disease, whereas 20 had coronary artery disease with chest pain at rest. After the cardiological evaluation, the patients underwent esophageal function testing by means of upper endoscopy, manometry, and 24-hour pH-monitoring.Overall, 10% of patients (2.5% in the coronary artery disease group) had evidence of endoscopic esophagitis, 46% of esophageal motor disorders (12% in the coronary artery disease group), and 10% abnormal pH-monitoring (1% in the coronary artery disease group).We report that the esophagus might be responsible for non-cardiac chest pain in patients with and without coronary artery disease. In our experience, esophageal motor disorders, and not an increased acid reflux, are the abnormalities most commonly found in these patients.

The aim of this study was to evaluate the risk of recurrent ischaemic cerebrovascular events (stroke or transient ischaemic attack (TIA)) in patients with patent foramen ovale (PFO) or atrial septal aneurysm (ASA) treated with different therapeutic regimens. We enrolled 86 patients aged 18-60 years with an unexplained ischaemic stroke or TIA referred to our inpatient department in the period May 1994-December 1999. Follow-up lasted until April 2003. Patients were excluded if the stroke or TIA was related to large-artery atherosclerosis, small artery occlusion, major cardiac sources of embolism or other uncommon causes. During a follow-up (mean+/-SD) of 64.1+/-28.8 months (range 8.1-105.6) a recurrent ischaemic cerebrovascular event occurred in 11/86 patients (12.8%) (5 TIA and 6 strokes). Eight events (4 TIA, 4 strokes) occurred in the 59 patients with PFO alone, three (1 TIA, 2 strokes) in the 21 with PFO plus ASA and none in the 6 patients with ASA alone. In the overall population the cumulative risk of recurrent stroke/TIA was 1.2% at 2 years, 5.5% at 4 years, 7.6% at 6 years and 23.6% at 8 years, and was similar in patients with PFO alone vs. patients with PFO plus ASA (9.0% vs. 6.1% at 6 years, 26.0% vs. 23.1% at 8 years; p0.05). Nine cerebral ischaemic events (4 TIA, 5 strokes) occurred in the 48 patients treated with antiplatelet drugs (7 in patients with PFO, 2 in patients with PFO plus ASA), and two (1 TIA, 1 stroke) in the 17 patients treated with oral anticoagulants (1 with PFO, 1 with PFO plus ASA). No events occurred in patients submitted to transcatheteral closure.

Patients with myocardial infarction without angiographically significant coronary artery lesions are considered, as a whole, to have a better prognosis. Different degrees of coronary involvement, within this wide group, may portend different degrees of risk. The aim of this study was to assess which clinical and angiographic covariates are more useful in defining the individual prognosis. METHLODS: We prospectively followed 53 consecutive patients admitted to our coronary care unit between 1985-1990 with myocardial infarction and a culprit lesion causingor = 50% angiographic luminal narrowing.Patients with normal angiograms (group A) were compared to those with minor parietal irregularities (group B) or discrete (or = 50%) stenosis (group C). Group A patients were younger, had a lower peak creatine kinase release, and a higher ejection fraction. After a median follow up of 125 +/- 32 months, group A patients had a 100% 10-year survival compared to 77 and 58% for group B and group C patients respectively (p = 0.01). At univariate analysis, ischemic events correlated with the severity of coronary lesions (while group A patients had no ischemic events at follow-up, events occurred in 46 and 50% of group B and C patients respectively; p = 0.003) and with ejection fraction, that was lower in patients with events (56.0 +/- 17 vs 67 +/- 11%, p = 0.006). Cardiac death, too, correlated with the type of coronary lesions (p = 0.03) and with lower ejection fraction (p = 0.004). By means of multivariate analysis, on the contrary, only the vessel morphology was predictive of ischemic events at follow-up (p = 0.02), while the only significant predictor of death was ejection fraction (p = 0.0012).Patients with myocardial infarction and strictly normal vessels have very few ischemic events at follow-up, and may be distinguished from both patients with non-significant lesions as well as those with minor angiographic irregularities. On the other hand, cardiac mortality correlates strongly and independently with a depressed ventricular function.

Fever of unknown origin is one of the most intriguing issues in clinical practice. One of the most feared diagnoses, especially in patients with known valvular disease, is endocarditis. The differential diagnosis of fever is often complicated by the clinical-pathological overlap between the systemic inflammatory response in different types of pathologies such as infectious, autoimmune or neoplastic disorders. We report a case of a patient presenting with fever, cutaneous nodules and malaise, with a known mitral valve prolapse and moderate regurgitation, in which the diagnosis of Wegener's granulomatosis was finally made.

Background . Morbidity and mortality of emergency repair of type A dissecting aneurysms of the aorta are high. This is an attempt to investigate the risk determinants of early and late results. Methods . A series of preoperative and operative variables were retrospectively collected from the clinical records of 291 patients operated on between January 1, 1979, and December 31, 1995. Risk factors for surgical death were investigated with univariate analysis and stepwise logistic regression. Follow-up was conducted between December 1995 and February 1996. Analysis of late results was conducted by means of actuarial survival curves (life method). After removing the surgical deaths, risk factors for late deaths were analyzed by a Cox model. Results . The in-hospital mortality rate was 36.1%. Significant independent determinants of operative or early death were preoperative shock, preoperative neurologic impairment, operation before 1986, perioperative bleeding, and prolonged clamping time. The 10-year survival rate was 36.9% ± 4.4%. Twenty-six patients required repeat operation. The long-term prognosis was significantly worse in patients who needed reoperation. Conclusions . Growing awareness of this disease and quicker diagnosis have increased the number of patients with acute dissection of the ascending aorta who are taken early to operation. This new challenge must be met by better preoperative support and intraoperative monitoring, and by surgical techniques that focus on lowering the rate of late complications, for which lifelong follow-up must be provided.

Patients presenting with ischemic chest pain and electrocardiographic evidence of global T-wave inversion are most frequently women with intact left ventricular function and no critical stenosis of major coronary vessels. Hence, this syndrome has a good immediate and long-term prognosis.

A 26-year-old woman developed critical stenosis of the left main coronary artery 10 years after radiation treatment for Hodgkins disease. She was operated on but died in the immediate postoperative period. At autopsy the pathological findings were indistinguishable from those described in "typical" atherosclerotic plaques.

OBJECTIVE--To define the clinical and angiographic features and the therapeutic problems in patients with coronary artery disease after therapeutic irradiation of the chest. DESIGN--An observational retrospective study. SETTING--The cardiac catheterisation laboratory, university medical school. PATIENTS--15 subjects (8 men and 7 women, aged 25-56 years, mean 44) examined in the cardiac catheterisation laboratory, who had significant coronary artery disease years after having radiation treatment to the chest and anterior mediastinum. In the early stages of the study angiography was performed because of typical symptoms of ischaemic heart disease. Later on it was performed because of a high index of suspicion in people with signs of extensive radiation heart damage. MAIN OUTCOME MEASURES--Clinical and electrocardiographic evidence of ischaemic heart disease; echocardiographic signs of pericardial, myocardial or valvar involvement; angiographic evidence of coronary arterial stenosis, with special attention to the ostia; haemodynamic and angiographic signs of pericardial, myocardial, and valvar disease. Survival and symptomatic and functional status were ascertained after medical or surgical treatment. RESULTS--The patients were relatively young and had no risk factors. Seven patients had no signs or symptoms of ischaemic heart disease. Ten patients had ostial stenosis, which was associated with extensive involvement of other cardiac structures in nine of them. Seven required surgical treatment for coronary artery disease. Two died, one at surgery and the other one six months later. Five patients had complications associated with irradiation. CONCLUSIONS--Coronary arterial disease can be reasonably ascribed to the effects of chest irradiation when the patients are young and free from risk factors, especially if the obstructions are ostial and there is important damage to other cardiac structures. In patients with damage to other cardiac structures angina and infarction are often absent and coronary angiography seems to be mandatory. Patients often require surgical treatment and postoperative complications are common.

We investigated the clinical, electrophysiological, haemodynamic and angiographic aspects of four patients (two men and two women, aged 31-46 years) who developed complete heart block 13-20 years after therapeutic irradiation of the chest for Hodgkin's disease. The initial cardiac symptom was syncope in three, effort intolerance in one. The electrocardiogram recorded third-degree atrioventricular block in three patients, right bundle branch block and posterior fascicular block in one. The electrophysiological study, performed in three cases, showed that the block was infranodal in two. Three patients had significant coronary arterial stenoses, that involved the ostia in two. All patients had mild-to-moderate aortic and mitral regurgitation. One patient had haemodynamic signs of constriction. Another patient had recurrent pericardial effusions. All had echocardiographic evidence of a thickened pericardium. Cardiac involvement can be extensive in patient with radiation-induced heart block. Because coronary artery disease can be particularly severe, coronary angiography appears to be warranted in such patients.

Retrosternal pain can be caused both by cardiac and esophageal disease. This work presents the results of cardiac and esophageal investigations in 55 patients, who had atypical chest pain. Isolated esophageal disease was found in 45% of the subjects while 14.5% had significant coronary arterial disease. Both diseases were found in 10.9% of the patients and neither disease in 29%. We conclude that esophageal disease is very frequent in patients with atypical chest pain but it does not always completely account for the symptoms. Such patients should, in our opinion, be submitted to an electrocardiographic stress test. If the result is positive or non-diagnostic, coronary cineangiography should be performed, irrespective of the results of esophageal investigations. If the electrocardiographic stress test is negative, coronary investigations can be deferred. Esophageal investigations can account for the symptoms in about half of such cases.

Patterns of motion of the aortic valve were analyzed with echocardiography in 9 patients with discrete subaortic stenosis and 31 patients with idiopathic hypertrophic subaortic stenosis, 22 with and 9 without a resting intraventricular pressure gradient. The intention was to determine whether the early systolic closure of the aortic valve was a sensitive indicator of a resting pressure gradient across the left ventricular outflow tract. All 9 patients with discrete subaortic stenosis and the 22 patients with idiopathic hypertrophic subaortic stenosis with a resting pressure gradient showed early systolic closure of the aortic valve; however, the 9 patients without a resting gradient had normal motion of the aortic valve. Measured values for O-ESC (the interval from the opening point of the aortic valve to the point of early systolic closure of the aortic valve) in 9 patients with discrete subaortic stenosis and in 22 with idiopathic hypertrophic subaortic stenosis averaged 0.05 ± 0.01 (standard deviation) second and 0.14 ± 0.04 second for each group, respectively (P < 0.01). Twelve patients with idiopathic hypertrophic subaortic stenosis underwent operation to alleviate left ventricular outflow tract obstruction. In eight of these patients the resting pressure gradient was completely abolished and early systolic closure of the aortic valve was no longer present. The results indicate that in idiopathic hypertrophic subaortic stenosis, early systolic closure of the aortic valve is recorded only when there is a significant intraventricular pressure gradient at rest. The time of occurrence of early systolic closure differentiated patients with discrete subaortic stenosis from those with idiopathic hypertrophic subaortic stenosis in all observations.

Summary Two cases of horseshoe lung are described; one was suspected and the other was diagnosed preoperatively. Both underwent successful surgical treatment. The embryology of this anomaly is briefly reviewed with reference to the closely related scimitar syndrome (anomalous venous return of right lung to inferior atriocaval junction). Diagnostic studies are discussed with stress on the need for a thorough functional evaluation of both the heart and lungs before the surgical indication is made.

We report four cases of so-called absent pulmonary valve syndrome associated with absence or anomalous origin of the left pulmonary artery. The fate of the patients with this condition appears to be affected mainly by the occurrence of pulmonary complications (three of our patients died of pulmonary causes, two of them after surgery). The proper timing of corrective surgery is still uncertain. Preoperative investigations should strive to obtain a clear-cut identification of the pulmonary arteries, particularly the left one or, in its absence, of the anomalous vascular supply to the lung. Right ventriculography, as employed by us, is insufficient. Pulmonary angiography, aortography and pulmonary vein "wedge" angiography may be needed.

A patient developed oliguria, peripheral edema, and dyspnea 5 days after mitral valve replacement. Chest roentgenogram and echocardiogram suggested pericardial effusion. Although pulsus paradoxus was absent, cardiac tamponade was suspected and subsequently confirmed during pericardiocentesis. Before pericardiocentesis right and left ventricular diastolic pressures were equal, as well as intrapericardial and right atrial pressures. The left ventricular cavity was reduced in size on the echocardiogram. Interventricular septal motion was normal. Pericardiocentesis normalized the hemodynamics and allowed the left ventricle to reexpand. Certain atypical features are tentatively explained as the combined effect of constrictive and effusive components caused by intrapericardial hemorrhage.

Sixty consecutive patients who were symptom free 2-12 months after an uncomplicated acute myocardial infarction underwent maximal treadmill exercise testing, radionuclide angiography before and during submaximal bicycle stress test, and coronary angiography. The results of the non-invasive procedures were compared with those of coronary angiography. The sensitivity and specificity of electrocardiogram stress test for detection of multivessel disease were 40% and 77% respectively. Failure of left ventricular ejection fraction to increase at least 5% with exercise identified 20 of the 25 patients with multivessel disease (sensitivity 80%) and 23 of the 35 patients with no additional coronary artery stenosis (specificity 66%). In patients with anterior Q waves the sensitivity was 78% and the specificity 50%, whereas in the presence of inferior Q waves these values were 81% and 87% respectively. Loss of left ventricle synchronicity during effort, as indicated by failure of the standard deviation of the phases to decrease during exercise, demonstrated a radionuclide angiography sensitivity of 80% (77% for anterior myocardial infarction and 81% for inferior myocardial infarction) and a specificity of 50% (33% for anterior myocardial infarction and 64% for inferior myocardial infarction). When the test was considered to be positive if either the ejection fraction or the standard deviation of the phases criteria were positive, the sensitivity was 100% and specificity 46% (30% for anterior myocardial infarction and 65% for inferior myocardial infarction). It is concluded that in patients who are free from angina 2-12 months after an episode of uncomplicated myocardial infarction, a simple exercise electrocardiogram cannot be relied upon to detect residual ischaemia. An abnormal ejection fraction response or an increased standard deviation of the phases during exercise nuclear angiography or both identified all the patients with multivessel disease. None of the patients in whom radionuclide angiographic criteria were negative had multivessel disease.

Recognition of thrombosis of a Bjork-Shiley aortic valve prosthesis 4 years after insertion in a patient was based upon sudden clinical deterioration, loss of prosthetic sounds, and development of new stenotic and regurgitant murmurs. Thrombotic fixation was confirmed by diagnostic alterations on the echocardiogram. All manifestations reverted to normal after successful surgical debridement of the prosthesis. Echocardiography is a valuable noninvasive adjunct in the differential diagnosis of prosthetic valve malfunction.

Second-degree intra-His bundle block is frequently of type I (Wenckebach periods) or 2:1. In this situation, the surface electrocardiogram does not permit distinction between intranodal (atrioventricular [A-V] and subnodal (intra-His) block. This study examined the value of bedside carotid sinus massage and atropine administration in diagnosing the site of block from the standard electrocardiogram in subjects with chronic A-V block and narrow QRS complexes. Fifteen patients had intra-His bundle block and 10 had intranodal block. The combination of two tests correctly located the site of block in 22 subjects, and was noncontributory in 3. Thirteen of the 15 intra-His bundle blocks and 9 of the 10 intranodal blocks were properly identified; in three cases the results were nondiagnostic, but no wrong diagnoses were made. The noninvasive bedside method of carotid sinus massage and the use of atropine permit both the localization and the determination of the type of block in the majority of cases of second degree A-V block and narrow QRS complexes. In a proper clinical context they can obviate the need for invasive electrophysiologic studies.

The late outcome in 226 patients who survived surgical repair of aortic coarctation was assessed 15-30 years after operation. Twenty six patients died during the follow up mainly from causes related to surgical repair or to associated cardiovascular anomalies. The survival rates of patients operated on between the ages of four and 20 years are 97%, 97%, 92% at 10, 20, and 30 years after operation. For patients operated on after the age of 20 the corresponding rates are 93%, 85%, and 68%. This difference is statistically significant from the fifteenth year of follow up onwards. The survival of patients operated on before the age of 20 is not significantly different from that of a comparable general Italian population. Recoarctation occurred in only 8% of patients who had end to end anastomosis, whereas it occurred in 35% of those who had other types of operation. Two thirds of the patients were hypertensive at the last visit. The actuarial curve shows that blood pressure was normal in most patients 5-10 years after operation, but 30 years after coarctation repair only 32% of patients are expected to be normotensive. Thus early repair of aortic coarctation appears to improve long term survival. Intervention in older patients and when blood pressure is high seem to be the most important predictors of late hypertension.

Summary A case is reported of ventricular extrasystolic rhythm showing the features of concealed ventricular bigeminy, and intermittent ventricular parasystolic rhythm, both arising from the same ectopic ventricular focus.

To aid in the study of coronary artery disease, 57 patients with complete left bundle branch block underwent clinical evaluation, treadmill exercise testing and cardiac catheterization. The patients were classified into two groups according to coronary anglographic findings: 30 patients with significant stenosis (70 percent or greater luminal narrowing) of at least one major vessel and 27 with no significant coronary artery disease. There was no difference in age, presenting symptoms or previous medical treatment between the two groups. There were more men in the group with coronary artery disease. Exercise-induced S-T changes were similar in the two groups; the sensitivity and specificity of these changes for the diagnosis of coronary artery disease were unacceptable irrespective of the criterion chosen. With additional S-T depression of either 1 or 2 mm below the baseline value, the predictive accuracy was only 53 percent. Combined exertional chest pain and 1 mm S-T depression increased the predictive accuracy of exercise testing to 71 percent. These data indicate that exercise-induced electrocardiographic changes do not facilitate detection of coronary artery disease in patients with complete left bundle branch block.

An 8 year-old boy had extensive electrophysiological evaluation of his recurrent supraventricular tachycardias. His ECG never showed delta waves but intracardiac stimulation and recording disclosed the following (1) eccentric retrograde atrial activation; (2) increased cycle length and retrograde conduction time following the development of right bundle-branch block; (3) constant retrograde conduction time for increasingly premature ventricular stimuli; (4) atrial captures by ventricular stimuli when the atrioventricular-His pathways were refractory; and (5) no delta waves upon stimulation of the atrial input site of the anomalous pathway. A diagnosis of reciprocating tachycardia involving retrograde conduction through an accessory pathway was made. Reciprocating tachycardias involving a unidirectional retrograde anomalous pathway can be easily misdiagnosed as atrioventricular node reentrant tachycardias if no evidence of preexcitation can be found, particularly if the anomalous pathway is on the right side. In order to exclude the participation of a concealed unidirectional anomalous pathway in a patient's reentry tachycardia, a complete map must be made of right and left atrial endocardial activity.

Today, total correction of tetralogy of Fallot is rarely performed in adults. In a 10-year period, 40 patients aged 20 to 67 years underwent intracardiac repair in our institution. Twenty-eight of them had had a palliative procedure 11 to 30 years earlier. Preoperatively, 23 patients were in New York Heart Association (NYHA) Functional Class II, 14 were in Class III, and 3 were in Class IV. Operative mortality was 2.5% (1/40). Follow-up ranged from 1 year to 11 years (average, 3 years). One patient died of a noncardiac cause 4 years after operation. Residual cardiac defects were observed in 4 patients. Postoperatively, 30 patients were in NYHA Functional Class I, 8 were in Class II, and 1 was in Class HI. Major ventricular arrhythmias were recorded in 7 (35%) of 20 patients. Radionuclide angiography demonstrated impaired right ventricular function in 8 patients. Left ventricular impairment was present in 2. Total correction of tetralogy of Fallot can be performed safely in adults with low mortality and good functional improvement. The incidence of residual cardiac defects is low. The long-term importance of impaired ventricular function and arrhythmias remains to be ascertained.

From January, 1979, to December, 1984, at the Cardiac Surgery Department of the University of Torino Medical School, major sternal wound infections developed in 48 (1.86%) of 2,579 consecutive patients. These patients underwent open-heart procedures through a midline sternotomy and survived long enough for infection to appear. Possible risk factors were evaluated by means of a multivariate analysis. For the group of patients, we considered age, sex, hospital environment (different locations of our surgical facilities over the years), interval between hospital admission and operation, antibiotic prophylaxis, type of surgical procedure, elective or emergency surgical procedure, reoperation, duration of surgical procedures, duration of cardiopulmonary bypass, amount of blood transfused, postoperative blood loss, chest reexploration, rewiring of a sterile sternal dehiscence, duration of mechanical ventilation, and days of treatment in the intensive care unit. Univariate analysis indicated that age, sex, type and mode of surgical procedure, antibiotic prophylaxis, and duration of mechanical ventilation were not significantly associated with wound infection. For all other predisposing factors, a p value of less than .05 was demonstrated. These variables were entered in a multiple stepwise logistic regression. Six emerged as significant: hospital environment (p = .0001), interval between admission and surgery (p = .041), reoperation (p less than .0001), blood transfusions (p = .031), early chest reexploration (p less than .0001), and sternal rewiring (p less than .0001). Contamination of patients may occur before, during, and after operation, and any kind of reintervention may predispose to wound infection.