Pitfalls in electrogram interpretation: Subcutaneous cardioverter defibrillator malfunction in Brugada syndrome.

A patient with Brugada syndrome implanted with subcutaneous implantable cardioverter defibrillator (S-ICD) had oversensing episodes treated with S-ICD shocks. Comparable artifacts were not evocable with S-ICD pocket manipulation. The fluoroscopy excluded S-ICD macroscopic damage. The device extraction revealed undamaged pulse generator and connector, but the lead was inappropriately tunneled under the sixth rib. Then the S-ICD malfunction was due to lead microscopic damage caused by the lead rubbing the rib surface.

Significant Drop in Right Atrial Pressure Does Not Influence Fractional Flow Reserve Coronary Assessment.

The effect of a highly elevated level of right atrial filling pressure on fractional flow reserve (FFR) measurement remains unclear. Transcatheter tricuspid valve intervention, a recently introduced option for inoperable or high-risk patients, represents a unique model of in-vivo physiology to investigate the eventual influence of central venous pressure on coronary FFR measurements. The case is reported of a patient with a degenerated tricuspid surgical bioprosthesis who underwent transcatheter tricuspid valve-in-valve replacement and concomitant coronary functional assessment with FFR. In an experimental model, the significant fall in right atrial pressure did not influence FFR measurements in the presence of angiographically proven mild coronary artery disease.

Dyspnea following thoracostomy closure after right pneumonectomy: An uncommon echocardiographic diagnosis and therapeutic approach.

Dyspnea and hypoxemia are common postoperative problems after pneumonectomy. One of the rarer causes of respiratory distress after right pneumonectomy is the development of a significant right-to-left shunt across a patent foramen ovale (PFO), which can evolve at a variable interval of time after the operation. We report here our experience with a patient who underwent right pneumonectomy, followed by several complications, and who presented severe dyspnea 7 months later, after the closure of a right thoracostomy. This report outlines the management of this challenging clinical condition; transesophageal echocardiography (TOE) provided a clear diagnosis and guided an effective percutaneous treatment.

Clinical outcomes of secondary prevention strategies for young patients with cryptogenic stroke and patent foramen ovale.

Background The aim of this study was to compare the immediate and long-term clinical outcomes of medical therapy and percutaneous patent foramen ovale (PFO) closure as secondary prevention strategies in patients younger than 55 years of age presenting with cryptogenic stroke and PFO. Methods Between January 2006 and April 2015, all patients with the diagnosis of cryptogenic stroke and PFO were analysed and prospectively followed. Stroke was confirmed in 159 out of 309 patients (51%). In the remaining cases, other neurological conditions were found and therefore excluded from further analysis. Patients received PFO closure or medical therapy on the basis of a pre-specified algorithm. Primary outcome was the assessment of recurrent ischaemic events at follow-up. Results Percutaneous PFO closure was performed in 77 patients (48%) and 82 (52%) were treated medically. Mean follow-up was 51.6 ± 34.8 months. Two ischaemic strokes occurred in the medical group only (2.4% vs 0%; P = 0.16) and no complications related to the invasive procedure were observed. Conclusions The diagnosis of stroke in patients with PFO could be confirmed in 50% of cases only, underlining the importance of a multidisciplinary evaluation of these patients. A very low ischaemic recurrence rate was observed in the medical therapy group, suggesting that a personalized treatment based on a prespecified diagnostic algorithm yields good clinical results irrespective of the treatment modality. Given the low number of recurrences, larger cohorts may be needed to prove significant differences.

Association between Beta1 -Adrenergic Receptor Polymorphism and Risk of ICD Shock in Heart Failure Patients.

BACKGROUND: Sympathetic activation in heart failure patients favors the development of ventricular arrhythmias, thus leading to an increased risk of sudden cardiac death. ß1 - and ß2 -adrenergic receptor polymorphisms have been linked to the risk of sudden death. Implantable cardioverter-defibrillators (ICD) are implanted in a large percentage of heart failure patients, and beyond preventing sudden cardiac death they provide a continuous monitoring of major ventricular arrhythmias and of their own interventions. We investigated whether functionally relevant ß1 - and ß2 -adrenergic receptor polymorphisms are associated with risk of ICD shocks, as evidenced in ICD memory. METHODS: 311 patients with systolic heart failure were enrolled, and number and timing of shocks in ICD memory were recorded. Four selected polymorphisms were determined: ß1 -adrenergic receptor polymorphisms Ser(49) Gly and Arg(389) Gly and ß2 -adrenergic receptor polymorphisms Arg(16) Gly and Gln(27) Glu. RESULTS: Only Ser(49) Gly was significantly correlated with time free from ICD shocks, both considering time to the first event in a Cox model (hazard ratio 2.117), and modeling repeated events with the Andersen-Gill method (hazard ratio 2.088). Gly allele carriers had a higher probability of ICD shock. The relationship remained significant even after adjusting for ejection fraction and beta-blocker dosage (hazard ratio 1.910). CONCLUSIONS: Data from our study suggest that the ß adrenoreceptor Gly 49 allele of the ß1 -adrenergic receptor Ser(49) Gly polymorphisms may increase the risk of ICD shock in patients with heart failure, independent of beta-blocker dosage.

Echocardiographically Derived Pulse Wave Velocity and Diastolic Dysfunction Are Associated with an Increased Incidence of Atrial Fibrillation in Patients with Systolic Heart Failure.

BACKGROUND: Chronic heart failure (CHF) is an established risk factor of atrial fibrillation (AF), but the prognostic value of cardiac and hemodynamic parameters in assessing the risk of developing AF among patients with CHF is less defined. METHODS AND RESULTS: We followed an outpatients cohort of CHF patients secondary to left ventricular (LV) systolic dysfunction, who were free of AF at baseline. All patients underwent clinical evaluation, comprehensive echocardiography, and blood drawing in the same morning. Aortic pulse wave velocity (aPWV), a measure of aortic stiffness, was determined by Doppler echocardiography. A total of 77 patients (age 63 ± 9 years; 79% male) with mean LVEF (34 ± 8%) formed the study population. Fifteen patients developed incidental AF. At baseline, CHF patients who developed AF during follow-up had higher E-wave velocity (75 ± 2 cm/sec vs. 60 ± 2 cm/sec; P = 0.02), higher difference duration between mitral and pulmonary vein A velocity (A'-A), (10 ± 35 msec vs. 43 ± 44 msec P = 0.02), aPWV (7.1 ± 2.6 vs. 5.3 ± 1.9 m/sec P = 0.004), and furosemide dosage (110 ± 145 mg vs. 49 ± 48 mg P = 0.01) than those remaining free from AF. The two groups of patients did not significantly differ in terms of NYHA, LV volumes, ejection fraction, left atrial volume, creatinine, hemoglobin, renin, epinephrine, amino-terminal propeptide of type III and I procollagens, ACE inhibitor, and ß-blocker dose (P > 0.1 for all). Notably, higher aPWV (P = 0.01) and longer A-A' duration (P = 0.04) were associated with an increased incidence of AF, independently of potential confounders. CONCLUSIONS: Increased aortic stiffness and LV diastolic dysfunction are strong predictors of new onset of AF among patients with systolic CHF.

Randomized Comparison of Xience V and Multi-Link Vision Coronary Stents in the Same Multivessel Patient With Chronic Kidney Disease (RENAL-DES) Study.

BACKGROUND: Percutaneous coronary interventions in patients with chronic kidney disease have shown suboptimal results. Drug-eluting stents (DES) might reduce the rate of target vessel revascularization in comparison with bare-metal stents (BMS) in patients with chronic kidney disease. However, given the multiple concomitant individual variables present in such patients, the comparison of neointimal growth after percutaneous coronary intervention is complex and difficult to assess. METHODS AND RESULTS: Randomized Comparison of Xience V and Multi-Link Vision Coronary Stents in the Same Multivessel Patient with Chronic Kidney Disease (RENAL-DES) was a prospective, randomized, multicenter study to directly compare the efficacy in the prevention of clinical restenosis of everolimus-eluting stent (Xience V) and BMS with an identical design (Multi-Link Vision), both implanted in the same patient with multivessel coronary artery disease and chronic kidney disease (estimated glomerular filtration rate <60 mL/min). The primary end point of the study was the ischemia-driven target vessel revascularization as detected with myocardial scintigraphy at 12 months. In 215 patients, 512 coronary vessels were successfully treated with the randomly assigned DES (n=257) or BMS (n=255). At 1 year, the rate of ischemia-driven target vessel revascularization for DES and BMS groups was 2.7% (95% confidence interval, 1.1%-5.6%) and 11.4% (95% confidence interval, 7.8% to 16%), respectively, P<0.001. For the multivariate analysis, independent predictors of the ischemia-driven target vessel revascularization were BMS implantation (odds ratio, 4.95; 95% confidence interval, 2.1-11.6; P<0.001) and vessel size (odds ratio, 0.32; 95% confidence interval, 0.1-0.7; P=0.006). CONCLUSIONS: This is the first randomized trial showing a reduction of clinical restenosis with a new-generation DES in comparison with a BMS of equal design, in patients who have chronic kidney disease with multivessel coronary artery disease. CLINICAL TRIAL REGISTRATION URL: http://clinicaltrials.gov. Unique identifier: NCT00818792.

Dynamic changes of repolarization abnormalities in takotsubo cardiomyopathy.

OBJECTIVE: This study analyses dynamicchangesin dispersion of ventricularrepolarization over the time course of takotsubo cardiomyopathy (TC), and their relationships with clinical features and life-threatening arrhythmias. METHODS AND RESULTS: All consecutive patients admitted to our division between January 2008 and December 2011 with a diagnosis of TC were analysed. Patients with prior myocardial infarction, symptoms-onset-to-admission time greater than 12 hours, an implanted pacemaker, or under treatment with drugs affecting QTinterval, were excluded. Standard 12-lead ECG recordings during the acute, subacute and chronic phases were collected for each patient. Twenty-four patients (23 women, 63 +/- 14 years) were includedin our analysis. Only one patient experienced ventricular arrhythmias (4.2%). Significant increases were observed in QT and QTc intervals (from 420 ?423 to 505 +/- 66 ms, P < 0.00001, and from 479 +/- 33 to 551 +/- 51 ms, P < 0.00001, respectively), QTdispersion (from 59 +/- 18 to 100 +/- 44 ms, P=0.0006), Tpeak-to-Tend (from 82 +/- 20 to 123 +/- 39 ms, P=0.00006) and Tpeak-to-Tend/QT (from 0.20 +/- 0.33 to 0.26 +/- 0.57, P=0.0003) during the subacute phase. All these parameters returned to baseline values in the chronic phase and did not show any significant differences between the acute and chronic phases. CONCLUSIONS: A marked increase in QTc, QT dispersion, Tpeak-to-Tend and Tpeak-to-Tend/QT was observed during the subacute phase; this increase was transient and reverted in allpatients before hospital discharge. Ofnote, these findings were not associated with an increased risk of life-threatening arrhythmias.

Mitral and aortic valve sclerosis/calcification and carotid atherosclerosis: results from 1065 patients.

This study assesses whether aortic valve sclerosis (AVS) and mitral annulus calcification (MAC) are associated with carotid artery atherosclerosis, independently of traditional cardiovascular risk factors. A total of 1065 patients underwent both echocardiography and carotid artery ultrasound scanning. AVS and MAC were defined as focal areas of increased echogenicity and thickening of the aortic leaflets or mitral valve annulus. Carotid artery atherosclerosis was defined as presence/absence of any atherosclerotic plaque or presence/absence of plaque >50 %. Of 1065 patients (65 ± 9 years; 38 % female) who comprised the study population, 642 (60 %) had at least one atherosclerotic plaque. AVS, but not mitral valve sclerosis; was associated with the presence of carotid atherosclerosis (odds ratio (OR) 1.9, 95 % confidence interval (CI) 1.2-3.9; P = 0.005) and the degree of carotid atherosclerosis (OR 2.1, 95 % CI 1.2-3.9; P = 0.01) in a multivariate model including age, gender, previous ischemic heart disease, hypertension, dyslipidemia, smoking, diabetes, family cardiovascular history, left ventricular size, mass, and ejection fraction, and left atrial size. AVS is a significant predictor of carotid atherosclerosis, independently of other cardiovascular clinical and echocardiographic risk factors.

Long-term clinical follow-up of the multicentre, randomized study to test immunosuppressive therapy with oral prednisone for the prevention of restenosis after percutaneous coronary interventions: Cortisone plus BMS or DES veRsus BMS alone to EliminAte Restenosis (CEREA-DES).

AIMS: To analyse the clinical outcome at 4 years in patients with coronary artery disease treated with bare metal stents (BMS) vs. BMS and oral prednisone, or drug-eluting stents (DES), all assuming similar adjunctive medical treatment. METHODS AND RESULTS: Five Italian hospitals enrolled 375 non-diabetic, ischaemic patients without contraindications to dual anti-platelet treatment or corticosteroid therapy in a randomized controlled study. The primary endpoint was the event-free survival of cardiovascular death, myocardial infarction, and recurrence of ischaemia needing repeated target vessel revascularization at 1 year, and this was significantly lower in the BMS group (80.8%) compared with the prednisone (88.0%) and DES group (88.8%, P = 0.04 and 0.006, respectively). The long-term analysis of the primary endpoint was a pre-specified aim of the trial, and was performed at 1447 days (median, IQ range = 1210-1641). Patients receiving BMS alone had significantly lower event-free survival (75.3%) compared with 84.1% in the prednisone group (HR: 0.447; 95% CI: 0.25-0.80, P = 0.007) and 80.6% in DES patients (HR: 0.519; 95% CI: 0.29-0.93, P = 0.03). Prednisone-treated patients did not develop new treatment-related clinical problems. Drug-eluting stents patients suffered more very late stent thrombosis as a cause of spontaneous myocardial infarction. The need for target vessel revascularization remained lower in the prednisone and DES groups (13.6 and 15.2%, respectively), compared with BMS (23.2%). CONCLUSIONS: The clinical benefits of prednisone compared with BMS only persisted almost unchanged at 4 years. Drug-eluting stents performed better than BMS at long-term, although the advantages observed at 1 year were in part attenuated because of the occurrence of very late stent thrombosis and late revascularizations. Clinical Trial NCT 00369356.

Spontaneous regression of a large rhabdomyoma of the interventricular septum.

We report the case of a large congenital rhabdomyoma of the interventricular septum diagnosed prenatally. The foetus was strictly monitored with ultrasound throughout the gestation period showing that the mass had increased in size until delivery. Despite the size of the mass, which appeared to occupy the right ventricle, the baby presented no symptoms both in utero and after birth. Serial echocardiography was used to document the regression of the mass in childhood.

Non-alcoholic fatty liver disease is associated with an increased prevalence of atrial fibrillation in hospitalized patients with type 2 diabetes.

NAFLD (non-alcoholic fatty liver disease) and AF (atrial fibrillation) are two pathological conditions that are highly prevalent in developed countries and share multiple risk factors. The relationship between NAFLD and AF in Type 2 diabetes is currently unknown. We studied a hospital-based sample of 702 patients with Type 2 diabetes discharged from our Division of Endocrinology during 2007-2011. The diagnosis of AF was confirmed in affected participants on the basis of ECGs and medical history by experienced cardiologists. NAFLD was defined by ultrasonographic detection of hepatic steatosis in the absence of other liver diseases. Of the 702 hospitalized patients included in the study, 514 (73.2%) of them had NAFLD and 85 (12.1%) had persistent or permanent AF. NAFLD was associated with an increased risk of prevalent AF {OR (odds ratio), 3.04 [95% CI (confidence interval), 1.54-6.02]; P<0.001}. Adjustments for age, sex, systolic BP (blood pressure), HbA1c, (glycated haemoglobin), estimated GFR (glomerular filtration rate), total cholesterol, electrocardiographic LVH (left ventricular hypertrophy), COPD (chronic obstructive pulmonary disease), and prior history of HF (heart failure), VHD (valvular heart disease) or hyperthyroidism did not attenuate the association between NAFLD and AF [adjusted OR, 5.88 (95% CI, 2.72-12.7); P<0.001]. In conclusion, our results show that ultrasound-diagnosed NAFLD is strongly associated with an increased prevalence of persistent or permanent AF in patients with Type 2 diabetes, independently of several clinical risk factors for AF. The potential impact of NAFLD on AF deserves particular attention, especially with respect to the implications for screening and surveillance strategies in the growing number of patients with NAFLD.

Late and very late coronary stent thrombosis: Intravascular ultrasound findings and associations with antiplatelet therapy.

BACKGROUND AND OBJECTIVE: Late and very-late stent occlusion remains a serious complication of coronary stenting. Despite their high anti-restenotic efficacy, drug-eluting stents (DES) have been associated to more late-thrombosis as compared to bare-metal stents (BMS). The aim of this study is to analyze the clinical presentation, angiographic, and intravascular ultrasound (IVUS) findings in patients with late or very late stent thrombosis and the relationship with the antiplatelet regimen. METHODS AND RESULTS: Between January 2007 and December 2011, 34 consecutive patients with clinical syndromes compatible with late or very-late stent occlusion were studied with IVUS during emergency catheterization; 25 had DES and 9 had BMS. Thrombotic total occlusion was more common in DES (16 = 64%) than BMS patients (2 = 22%; P = 0.02) and ST-segment elevation myocardial infarction was the predominant clinical presentation in the former group (60% compared to 22%; P = 0.05). The time elapsed between implantation and failure was much longer in DES patients: 33 ± 22 versus 17 ± 14 months for BMS; P = 0.05. IVUS analysis showed a higher incidence of incomplete stent apposition (ISA) in the DES group compared to the BMS group (56% vs. 11%; P = 0.005) and in-stent plaque rupture was the most common finding in the BMS group (78%). In DES group, ISA was found more frequently in patients still under dual antiplatelet therapy (71%). CONCLUSIONS: Very late DES failure often causes ST-elevation myocardial infarction; these very late events may not correlate with the interruption of antiplatelet therapy. Severe, late acquired ISA belongs to DES and is frequently observed in patients with very late DES thrombosis.

Aortic stiffness: an old concept for new insights into the pathophysiology of functional mitral regurgitation.

Functional mitral regurgitation (FMR) is thought to be linked with ventricular afterload. However, the relation between aortic stiffness, which is a main determinant of ventricular afterload, and quantitatively assessed mitral regurgitation is unknown. A total of 175 patients (age 61 ± 13; 85 % male) with left ventricular (LV) systolic dysfunction were studied consecutively. Left ventricular volumes, ejection fraction, and LV outflow tract stroke volume were measured. Aortic pulse wave velocity (PWV), a known marker of aortic stiffness, was determined using Doppler flow recordings as the distance (d) traveled by the pulse wave, measured over the body surface as the distance between the two recording sites, divided by the time (t) taken by the pulse wave to travel from the descending aorta to the abdominal aorta. Mitral effective regurgitant orifice (ERO), regurgitant volume (RV), and fraction (RF) were measured using the proximal isovelocity surface area method. The mean PWV was 6.0 ± 3.5 m/s (range 2.6-25). PWV was significantly associated with ERO (r = 0.35; p < 0.0001), RV (r = 0.36; p < 0.0001) RF (p = 0.41; p < 0.0001). The association of PWV with each variable of mitral regurgitation was independent of LV volume, cardiac output, and systemic vascular resistance. Aortic stiffness is an important determinant of the severity of FMR. Aortic stiffness should be considered an important therapeutic target in patients with LV dysfunction in order to ameliorate both LV systolic and diastolic function and mitral regurgitation.

Huge ST elevation and ventricular arrhythmias in vasospastic angina diagnosed by Holter monitoring.

We report a case of a 67year old man with a recent history of sporadic chest pain and palpitations. After a normal, maximal stress test he underwent 24-hours Holter monitoring that showed two short periods of huge ST segment elevation associated with non sustained ventricular tachycardia and chest pain, referable to coronary spasm. Coronary angiogram showed significant atherosclerotic stenosis in the right coronary artery treated by angioplasty and bare metal stent implantation. Holter monitoring is a non-invasive and low-cost examination that can give valuable informations; it should be reserved for selected patients when vasospastic angina is suspected.

Sex-specific benefits of sirolimus-eluting stent on long-term outcomes in patients with ST-elevation myocardial infarction undergoing primary percutaneous coronary intervention: insights from the Multicenter Evaluation of Single High-Dose Bolus Tirofiban Versus Abciximab With Sirolimus-Eluting Stent or Bare-Metal Stent in Acute Myocardial Infarction Study trial.

OBJECTIVES: We assessed the relation between female sex and sirolimus-eluting stent (SES) use on long-term outcomes in acute myocardial infarction. BACKGROUND: There are no data on sex-specific differences in long-term benefit of SES use compared with bare-metal stent (BMS) use among patients undergoing primary percutaneous coronary interventions. METHODS: We performed a post hoc analysis of the MULTISTRATEGY trial. Hazard ratios (HRs) of events with 95% CI for sex and stent type were computed using Cox proportional regression with adjustment for confounders. RESULTS: A total of 744 patients, 64 years old (55-73 years old), 179 (24.1%) women, were enrolled. After a follow-up of 1,080 days, SES use was associated with a significant reduction of major adverse cardiovascular events, that is, the composite of all-cause death, reinfarction, or clinically driven target vessel revascularization (TVR) (13.9% vs 23.6%, adjusted HR 0.62, 95% CI 0.41-0.94, P = .026) and of TVR (6.1% vs 15.1%, adjusted HR 0.35, 95% CI 0.19-0.63, P < .001) in men. Conversely, SES use was not associated to a better outcome among women (major adverse cardiovascular events 21.9% in SES vs 18.2% in the BMS group, adjusted HR 1.27, 95% CI 0.53-3.02, P = .59; TVR 6.6% vs 9.1%, adjusted HR 0.62, 95% CI 0.17-2.21, P = .46). CONCLUSIONS: In this analysis, the clinical benefit of SES use, over BMS, at 3-year follow-up was restricted to men and was not observed among women.

Comparison of serum creatinine and cystatin C for early diagnosis of contrast-induced nephropathy after coronary angiography and interventions.

BACKGROUND: The diagnostic accuracy of serum creatinine and cystatin C (Cys) as early predictors of contrast-induced nephropathy (CIN) has been debated. We investigated the diagnostic sensitivities, diagnostic specificities, and variations from baseline for serum creatinine and Cys in CIN. METHODS: We prospectively evaluated 166 patients at risk for CIN at baseline, and at 12, 24, and 48 h after exposure to contrast media. CIN occurred in 30 patients (18%). Changes (Δ) compared to baseline in serum creatinine and Cys were evaluated at the predefined time points. ROC curve analysis was performed for the Δ 12-h basal serum creatinine and Cys. RESULTS: The Δ serum creatinine at 12 h from baseline was the earliest predictor of CIN [area under the ROC curve (AUC) = 0.80; P < 0.001]. The Δ serum creatinine 15% variation [0.15 mg/dL (13.2 µmol/L)] yielded 43% diagnostic sensitivity and 93% diagnostic specificity. The ΔCys at 12 h from baseline performed significantly worse than serum creatinine (AUC = 0.48; P = 0.74). CONCLUSIONS: Variations from the serum creatinine baseline offer better diagnostic accuracy for predicting CIN at an earlier stage than similar variations in Cys. An additional diagnostic value of Cys over the determination of serum creatinine in the setting of CIN was not observed.

Influence of myocardial fibrosis on left ventricular hypertrophy in patients with symptomatic severe aortic stenosis.

AIM: It was the aim of our study to determine whether myocardial fibrosis influences physiologic or non-physiologic left ventricular (LV) hypertrophy in severe aortic stenosis. METHODS: Myocardial fibrosis was evaluated using specimens taken from the ventricular septum in 79 patients submitted to aortic valve replacement because of symptomatic aortic stenosis. Patients were considered to have physiologic LV hypertrophy if end-systolic wall stress, evaluated by echocardiography, was <90 kdyn/cm(2), while those with end-systolic wall stress >90 kdyn/cm(2) were considered to have non-physiologic hypertrophy. RESULTS: Fibrosis tissue mass index was significantly inversely related with LV fractional shortening and directly related with LV diastolic and systolic diameter and LV mass index (LVMI). Patients with non-physiologic hypertrophy (n = 24) had a higher LVMI due to larger LV diastolic and systolic diameters with thinner wall, resulting in lower relative wall thickness. These patients had a higher fibrosis tissue mass index and impaired LV systolic and diastolic functions, as suggested by lower LV fractional shortening and higher mean wedge pressure. At follow-up of 7.4 ± 2.1 months, the LVMI and New York Heart Association class remained higher in patients with non-physiologic hypertrophy. CONCLUSIONS: Our study suggests a different quality of hypertrophies in patients with aortic stenosis, where myocardial fibrosis seems to be the critical abnormality that differentiates adaptive from maladaptive response to increased afterload.

Cardiac resynchronization therapy or sequential pacing in failing Mustard?

The atrial switch (Mustard, Senning procedures) was one of the treatments of choice for repair of transposition of the great arteries from the early 1960s to the mid-1980s. A significant proportion of patients with atrial switch develops systemic (right) ventricular failure. A series of surgical therapeutic options exists to manage cardiac failure in this setting, and, more recently proposed, cardiac resynchronization therapy. We describe case report of a 30-year-old woman with congenital heart disease (CHD) and previous Mustard procedure who underwent upgrading from single chamber to dual-chamber pacemaker. The narrower native QRS did not correlate with a better synchrony status nor with a better cardiac output. Functional evaluation confirmed a better performance in DDD mode with short atrioventricular delay and broad QRS. Some echocardiographic and electrocardiographic parameters, such as ejection fraction and QRS duration, well established in adults' heart for selection of candidates to cardiac resynchronization therapy, are much less studied in CHD. Postoperative CHD may provide unique patterns of asynchrony with poorly predictable hemodynamic outcome.