Immunodeficiency is prevalent in congenital heart disease and associated with increased risk of emergency admissions and death.

AIMS: To provide population-based data on the prevalence and clinical significance of immune deficiency syndromes (IDS) associated with congenital heart disease (CHD). METHODS AND RESULTS: Utilizing administrative German Health System data the prevalence of increased susceptibility to infection (ISI) or confirmed IDS was assessed in CHD patients and compared with an age-matched non-congenital control group. Furthermore, the prognostic significance of IDS was assessed using all-cause mortality and freedom from emergency hospital admission. A total of 54 449 CHD patients were included. Of these 14 998 (27.5%) had ISI and 3034 (5.6%) had a documented IDS (compared with 2.9% of the age-matched general population). During an observation period of 394 289 patient-years, 3824 CHD patients died, and 31 017 patients experienced a combined event of all-cause mortality or emergency admission. On multivariable Cox proportional-hazard analysis, the presence of ISI [hazard ratio (HR): 2.14, P < 0.001] or documented IDS (HR: 1.77, P = 0.035) emerged as independent predictors of all-cause mortality. In addition, ISI and confirmed IDS were associated with a significantly higher risk of emergency hospital admission (P = 0.01 for both on competing risk analysis) during follow-up. CONCLUSION: Limited immune competence is common in CHD patients and associated with an increased risk of morbidity and mortality. This highlights the need for structured IDS screening and collaboration with immunology specialists as immunodeficiency may be amenable to specific therapy. Furthermore, studies are required to assess whether IDS patients might benefit from intensified antibiotic shielding or tailored prophylaxis.

Lack of specialist care is associated with increased morbidity and mortality in adult congenital heart disease: a population-based study.

AIMS: The aim of this study was to provide population-based data on the healthcare provision for adults with congenital heart disease (ACHD) and the impact of cardiology care on morbidity and mortality in this vulnerable population. METHODS AND RESULTS: Based on administrative data from one of the largest German Health Insurance Companies, all insured ACHD patients (<70 years of age) were included. Patients were stratified into those followed exclusively by primary care physicians (PCPs) and those with additional cardiology follow-up between 2014 and 2016. Associations between level of care and outcome were assessed by multivariable/propensity score Cox analyses. Overall, 24 139 patients (median age 43 years, 54.8% female) were included. Of these, only 49.7% had cardiology follow-up during the 3-year period, with 49.2% of patients only being cared for by PCPs and 1.1% having no contact with either. After comprehensive multivariable and propensity score adjustment, ACHD patients under cardiology follow-up had a significantly lower risk of death [hazard ratio (HR) 0.81, 95% confidence interval (CI) 0.67-0.98; P = 0.03) or major events (HR 0.85, 95% CI 0.78-0.92; P < 0.001) compared to those only followed by PCPs. At 3-year follow-up, the absolute risk difference for mortality was 0.9% higher in ACHD patients with moderate/severe complexity lesions cared by PCPs compared to those under cardiology follow-up. CONCLUSION: Cardiology care compared with primary care is associated with superior survival and lower rates of major complications in ACHD. It is alarming that even in a high resource setting with well-established specialist ACHD care approximately 50% of contemporary ACHD patients are still not linked to regular cardiac care. Almost all patients had at least one contact with a PCP during the study period, suggesting that opportunities to refer patients to cardiac specialists were missed at PCP level. More efforts are required to alert PCPs and patients to appropriate ACHD care.

Maternal and neonatal complications in women with congenital heart disease: a nationwide analysis.

AIMS: The aim of this study was to provide population-based data on maternal and neonatal complications and outcome in the pregnancies of women with congenital heart disease (CHD). METHODS AND RESULTS: Based on administrative data from one of the largest German Health Insurance Companies (BARMER GEK, ∼9 million members representative for Germany), all pregnancies in women with CHD between 2005 and 2018 were analysed. In addition, an age-matched non-CHD control group was included for comparison and the association between adult CHD (ACHD) and maternal or neonatal outcomes investigated. Overall, 7512 pregnancies occurred in 4015 women with CHD. The matched non-CHD control group included 6502 women with 11 225 pregnancies. Caesarean deliveries were more common in CHD patients (40.5% vs. 31.5% in the control group; P < 0.001). There was no excess mortality. Although the maternal complication rate was low in absolute terms, women with CHD had a significantly higher rate of stroke, heart failure and cardiac arrhythmias during pregnancy (P < 0.001 for all). Neonatal mortality was low but also significantly higher in the ACHD group (0.83% vs. 0.22%; P = 0.001) and neonates to CHD mothers had low/extremely low birth weight or extreme immaturity (<0.001) or required resuscitation and mechanical ventilation more often compared to non-CHD offspring (P < 0.001 for both). On multivariate logistic regression maternal defect complexity, arterial hypertension, heart failure, prior fertility treatment, and anticoagulation with vitamin K antagonists emerged as significant predictors of adverse neonatal outcome (P < 0.05 for all). Recurrence of CHD was 6.1 times higher in infants to ACHD mothers compared to controls (P < 0.0001). CONCLUSIONS: This population-based study illustrates a reassuringly low maternal mortality rate in a highly developed healthcare system. Nevertheless, maternal morbidity and neonatal morbidity/mortality were significantly increased in women with ACHD and their offspring compared to non-ACHD controls highlighting the need of specialized care and pre-pregnancy counselling.

Aortic valve replacement in a patient with extra-anatomic aortic and subclavian bypasses. / Zamena aortal'nogo klapana u patsienta s ékstraanatomicheskimi aortal'nymi i podkliuchichnymi shuntami.

The patient was 62-year-old male who successfully underwent an anatomic repair of aortic coarctation at the age of 15 and an extra-anatomic ascending aorta- to-descending aorta bypass (graft size 20 mm) and an extra-anatomic ascending aorta to the left subclavian artery bypass (graft size 10 mm) at the age of 49. As he grew older he started presenting clinical symptoms of aortic stenosis. The echocardiogram showed a bicuspid aortic valve with severe stenosis and a mean gradient of 65 mm Hg. Despite the high surgical risk, we decided to perform an open valve replacement with installing the CPB prior to re-sternotomy with a simultaneous cannulation of the axillary and femoral arteries by reason of interrupted descending aorta. The postoperative course was uneventful.

Skeletal dysplasia in a consanguineous clan from the island of Nias/Indonesia is caused by a novel mutation in B3GAT3.

We describe a large family with disproportionate short stature and bone dysplasia from Nias in which we observed differences in severity when comparing the phenotypes of affected individuals from two remote branches. We conducted a linkage scan in the more severely affected family branch and determined a critical interval of 4.7 cM on chromosome 11. Sequencing of the primary candidate gene TBX10 did not reveal a disease-causing variant. When performing whole exome sequencing we noticed a homozygous missense variant in B3GAT3, c.419C>T [p.(Pro140Leu)]. B3GAT3 encodes ß-1,3-glucuronyltransferase-I (GlcAT-I). GlcAT-I catalyzes an initial step of proteoglycan synthesis and the mutation p. (Pro140Leu) lies within the donor substrate-binding subdomain of the catalytic domain. In contrast to the previously published mutation in B3GAT3, c.830G>A [p.(Arg277Gln)], no heart phenotype could be detected in our family. Functional studies revealed a markedly reduced GlcAT-I activity in lymphoblastoid cells from patients when compared to matched controls. Moreover, relative numbers of glycosaminoglycan (GAG) side chains were decreased in patient cells. We found that Pro140Leu-mutant GlcAT-I cannot efficiently transfer GlcA to the linker region trisaccharide. This failure results in a partial deficiency of both chondroitin sulfate and heparan sulfate chains. Since the phenotype of the Nias patients differs from the Larsen-like syndrome described for patients with mutation p.(Arg277Gln), we suggest mutation B3GAT3:p.(Pro140Leu) to cause a different type of GAG linkeropathy showing no involvement of the heart.

Occurrence and Outcome of Infective Endocarditis after Surgical Compared to Transcatheter Pulmonary Valve Implantation in Congenital Heart Disease.

Background: Conflicting data exist on the occurrence and outcome of infective endocarditis (IE) after pulmonary valve implantation. Objectives: This study sought to assess the differences between transcatheter pulmonary valve implantation (TPVI) and surgical pulmonary valve replacement (SPVR). Methods: All patients ≥ 4 years who underwent isolated pulmonary valve replacement between 2005 and 2018 were analyzed based on the data of a major German health insurer (≈9.2 million insured subjects representative of the German population). The primary endpoint was a composite of IE occurrence and all-cause death. Results: Of 461 interventions (cases) in 413 patients (58.4% male, median age 18.9 years [IQR 12.3-33.4]), 34.4% underwent TPVI and 65.5% SPVR. IE was diagnosed in 8.0% of cases during a median follow-up of 3.5 years. Risk for IE and all-cause death was increased in patients with prior IE (p < 0.001), but not associated with age (p = 0.50), sex (p = 0.67) or complexity of disease (p = 0.59). While there was no difference in events over the entire observational time period (p = 0.22), the time dynamics varied between TPVI and SPVR: Within the first year, the risk for IE and all-cause death was lower after TPVI (Hazard Ratio (HR) 95% CI 0.19 (0.06-0.63; p = 0.006) but increased over time and exceeded that of SPVR in the long term (HR 10.07 (95% CI 3.41-29.76; p < 0.001). Conclusions: Patients with TPVI appear to be at lower risk for early but higher risk for late IE, resulting in no significant difference in the overall event rate compared to SPVR. The results highlight the importance of long-term specialized care and preventive measures after both interventions.

Pacemaker Implantation for Low-Grade Conduction Abnormalities After Balloon-Expandable Transcatheter Aortic Valve Implantation.

INTRODUCTION: A frequent complication after TAVI are postinterventional conduction abnormalities requiring permanent pacemaker implantation. In this study, we analyzed the characteristics of borderline conduction abnormalities leading to pacemaker implantation and the resulting ventricular pacing amounts. METHODS AND RESULTS: All patients who underwent balloon-expandable TAVI between 2014 and 2019 in our tertiary center were analyzed in a retrospective manner. One hundred and sixty-five patients of 1083 TAVI-patients developed postinterventional conduction abnormalities leading to pacemaker implantation. Of these 19 (11.5%) did not represent a clear indication for cardiac pacing according to current European guidelines. Patient characteristics, underlying conduction abnormalities, and the temporal change of ventricular pacing percentages at 24 h and 6 weeks after pacemaker implantation were analyzed. The dominating borderline conduction abnormalities leading to pacemaker implantation were new-onset persisting bundle-branch-blocks and new first-degree AV-blocks with progression of AV-delay. While pacemaker implantation was safe and without severe complications in all cases, only 6 of 19 patients had high pacing amounts (95%-100%) after 24 h while 11 patients had low to no pacing amounts (0%-5%). After 6 weeks, 8 patients showed decreasing pacing amounts, no patient had an increasing amount of ventricular pacing and all patients had an intrinsic ventricular rhythm > 30/min. CONCLUSION: In our cohort of 1038 TAVI patients, 19 patients underwent PMI for borderline CAs (11.5% of all PMI). Of these, only 2 patients had high pacing amounts after 6 weeks. The risk of complete persisting heart block in these patients is very low. Furthermore, algorithms to reduce ventricular pacing are highly effective to avoid ventricular pacing whenever reasonable.

Utility of Coronary Computed Tomography Angiography in Patients Undergoing Transcatheter Aortic Valve Implantation: A Meta-Analysis and Meta-Regression Based on Published Data from 7458 Patients.

BACKGROUND: Coronary CT angiography (CCTA) may detect coronary artery disease (CAD) in transcatheter aortic valve implantation (TAVI) patients and may obviate invasive coronary angiography (ICA) in selected patients. We assessed the diagnostic accuracy of CCTA for detecting CAD in TAVI patients based on published data. METHODS: Meta-analysis and meta-regression were performed based on a comprehensive electronic search, including relevant studies assessing the diagnostic accuracy of CCTA in the setting of TAVI patients compared to ICA. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV), were calculated on a patient and per segment level. RESULTS: Overall, 27 studies (total of 7458 patients) were included. On the patient level, the CCTA's pooled sensitivity and NPV were 95% (95% CI: 93-97%) and 97% (95% CI: 95-98%), respectively, while the specificity and PPV were at 73% (95% CI: 62-82%) and 64% (95% CI: 57-71%), respectively. On the segmental coronary vessel level, the sensitivity and NPV were 90% (95% CI: 79-96%) and 98% (95% CI: 97-99%). CONCLUSIONS: This meta-analysis highlights CCTA's potential as a first-line diagnostic tool although its limited PPV and specificity may pose challenges when interpreting heavily calcified arteries. This study underscores the need for further research and protocol standardization in this area.

Comparison of two self-expanding transcatheter heart valves for degenerated surgical bioprostheses: the AVENGER multicentre registry.

BACKGROUND: There is a lack of comparative data on transcatheter aortic valve implantation (TAVI) in degenerated surgical prostheses (valve-in-valve [ViV]). AIMS: We sought to compare outcomes of using two self-expanding transcatheter heart valve (THV) systems for ViV. METHODS: In this retrospective multicentre registry, we included consecutive patients undergoing transfemoral ViV using either the ACURATE neo/neo2 (ACURATE group) or the Evolut R/PRO/PRO+ (EVOLUT group). The primary outcome measure was technical success according to Valve Academic Research Consortium (VARC)-3. Secondary outcomes were 30-day all-cause mortality, device success (VARC-3), coronary obstruction (CO) requiring intervention, rates of severe prosthesis-patient mismatch (PPM), and aortic regurgitation (AR) ≥moderate. Comparisons were made after 1:1 propensity score matching. RESULTS: The study cohort comprised 835 patients from 20 centres (ACURATE n=251; EVOLUT n=584). In the matched cohort (n=468), technical success (ACURATE 92.7% vs EVOLUT 88.9%; p=0.20) and device success (69.7% vs 73.9%; p=0.36) as well as 30-day mortality (2.8% vs 1.6%; p=0.392) were similar between the two groups. The mean gradients and rates of severe PPM, AR ≥moderate, or CO did not differ between the groups. Technical and device success were higher for the ACURATE platform among patients with a true inner diameter (ID) >19 mm, whereas a true ID ≤19 mm was associated with higher device success - but not technical success - among Evolut recipients. CONCLUSIONS: ViV TAVI using either ACURATE or Evolut THVs showed similar procedural outcomes. However, a true ID >19 mm was associated with higher device success among ACURATE recipients, whereas in patients with a true ID ≤19 mm, device success was higher when using Evolut.

Surgical Considerations for Treatment of Fungal Homograft Endocarditis in Re-re-re-re-do.

Fungal endocarditis is associated with high surgical mortality rates. Advanced expertise is required for surgical treatment of this serious condition. In the present report, we describe the homograft replacement in a beating heart during re-re-re-re-do in a 29-year-old female patient with fungal endocarditis. The previous operations included Fallot correction at the age of 1 year, Contegra graft implantation in the right ventricular outflow tract (RVOT) due to severe pulmonary insufficiency, homograft implantation in pulmonary position due to Contegra endocarditis, and on-pump pericardial defect closure after homograft injury during sternal rewiring following wound infection.

Thrombembolic occlusion of crural arteries following transcatheter aortic valve implantation--successful endovascular recanalization using a thrombus aspiration device.

Transcatheter aortic valve implantation (TAVI) has become an increasingly used alternative to conventional surgical valve replacement in patients with severe aortic valve stenosis (AS) and high operative risk. We here describe a case of a TAVI performed in local anesthesia causing intraprocedural thromboembolic occlusion of non-stenotic crural arteries and its immediate successful therapeutic management by means of endovascular recanalization using a thrombus aspiration device.

Thyroid Dysfunction under Amiodarone in Patients with and without Congenital Heart Disease: Results of a Nationwide Analysis.

Background: Amiodarone has a profound adverse toxicity profile. Large population-based analyses quantifying the risk of thyroid dysfunction (TD) in adults with and without congenital heart disease (ACHD) are lacking. Methods: All adults registered with a major German health insurer (≈9.2 million members) with amiodarone prescriptions were analyzed. Occurrence of amiodarone-associated TD was assessed. Results: Overall, 48,891 non-ACHD (37% female; median 73 years) and 886 ACHD (34% female; median 66 years) received amiodarone. Over 184,787 patient-years, 10,875 cases of TD occurred. The 10-year risk for TD was 38% in non-ACHD (35% ACHD). Within ACHD, compared to amiodarone-naïve patients, the hazard ratio (HR) for TD was 3.9 at 4 years after any amiodarone exposure. TD was associated with female gender (HR 1.42, p < 0.001) and younger age (HR 0.97 per 10 years, p = 0.009). Patients with congenital heart disease were not at increased risk (HR 0.98, p = 0.80). Diagnosis of complex congenital heart disease, however, was a predictor for TD (HR 1.56, p = 0.02). Amiodarone was continued in 47% of non-ACHD (38% ACHD), and 2.3% of non-ACHD (3.5% ACHD) underwent thyroid surgery/radiotherapy. Conclusions: Amiodarone-associated TD is common and comparable in non-ACHD and ACHD. While female gender and younger age are predictors for TD, congenital heart disease is not necessarily associated with an elevated risk.

Asymptomatic aortic stenosis: when to operate?

Although there is general agreement that symptomatic aortic stenosis (AS) requires urgent surgery, it remains controversial when to operate on asymptomatic but nevertheless severe AS. Current practice guidelines recommend valve replacement in patients in asymptomatic when the systolic left ventricular function is found to be reduced without other explanation. Otherwise, surgery should be or at least may be considered (recommendation classes IIa or IIb) in asymptomatic patients with severe AS and an abnormal exercise test, a high likelihood of rapid progression, or very severe AS in the presence of low surgical risk. This article reviews recent publications evaluating early elective surgery versus watchful waiting as recommended by current guidelines. The second focus of this review is on new insights on predictors of outcome in asymptomatic AS that may improve timing of surgery and therefore deserve to be considered in future recommendations for the treatment of this difficult patient group.

Frequency, Mortality, and Predictors of Adverse Outcomes for Endocarditis in Patients with Congenital Heart Disease: Results of a Nationwide Analysis including 2512 Endocarditis Cases.

BACKGROUND: Infective endocarditis (IE) represents a major complication in patients with congenital heart disease (CHD) and is associated with high morbidity and mortality. The aim of this study was to analyse the frequency and outcome of IE in contemporary CHD patients based on all IE hospital admissions in Germany over a 10-year period. METHODS: Based on data of all hospital admissions in Germany from 2009 to 2018, we identified all CHD cases with a diagnosis of IE. The data contained information on patient demographics, diagnoses, surgical procedures, and mortality. The primary endpoint of the study was endocarditis-associated mortality as well as major adverse events (defined as death or myocardial infarction, stroke, pulmonary embolism, sepsis, renal dialysis, resuscitation, or intubation). RESULTS: Overall, 309,245 CHD inpatient cases were included in the analysis (underlying heart defects of simple complexity 55%, moderate complexity 23%, and complex heart defects 22%, respectively). Of those, 2512 (0.8% of all inpatient cases) were treated for IE. The mortality rate of IE inpatient cases was 6% with a major adverse events rate of 46%, and 41.5% of cases required surgical intervention. The overall IE associated mortality was lower in adult CHD cases compared to the 153,242 in adult IE cases without CHD (7.1% vs. 16.1%, p < 0.001). After adjustments using multivariable logistic regression analysis, the presence or complexity of CHD was not associated with the outcomes. Meanwhile, age, male sex, and co-morbidities emerged as significant predictors of adverse outcomes. CONCLUSIONS: IE accounts for a minority of CHD related hospitalizations but remains a deadly disease, and major adverse events are common in this setting. Due to different demographic and co-morbidity spectrums, adult CHD patients tend to have better survival prospects when compared to non-CHD IE patients. Acquired co-morbidities emerged as the main predictors of adverse outcomes.

Mortality and morbidity in patients with congenital heart disease hospitalised for viral pneumonia.

OBJECTIVES: Data on the clinical outcome of patients with congenital heart disease (CHD) affected by severe viral pneumonia are limited. We analysed morbidity and mortality of viral pneumonia and evaluated the association between medical conditions, medication, vaccination and outcome specifically in patients with CHD requiring hospitalisation for viral pneumonia. METHODS: Based on data from one of Germany's largest health insurers, all cases of viral pneumonia requiring hospital admission (2005-2018) were studied. Mortality, and composites of death, transplantation, mechanical circulatory support, ventilation or extracorporeal lung support served as endpoints. RESULTS: Overall, 26 262 viral pneumonia cases occurred in 24 980 patients. Of these, 1180 cases occurred in patients with CHD. Compared with patients without CHD, mortality rate was elevated in patients with CHD. As a group, patients with CHD aged 20-59 years even exceeded mortality rates in patients without CHD aged >60 years. No mortality was observed in patients with CHD with simple defects <60 years of age without associated cardiovascular risk factors. On multivariable logistic regression analysis, age, CHD complexity, chromosomal anomalies, cardiac medication, use of immunosuppressants and absence of vaccination for influenza emerged as risk factors of adverse outcome. CONCLUSIONS: We present timely data on morbidity and mortality of severe viral pneumonia requiring hospital admission in patients with CHD. Need for mechanical ventilation and risk of death in CHD increase early in life, reaching a level equivalent to non-CHD individuals >60 years of age. Our data suggest that except for patients with isolated simple defects, patients with CHD should be considered higher-risk individuals when faced with severe viral pneumonia.

Prospective, multicentre validation of a simple, patient-operated electrocardiographic system for the detection of arrhythmias and electrocardiographic changes.

AIMS: Electrocardiographic changes, e.g. arrhythmias causing syncope or palpitations, are often transient and therefore difficult to diagnose. Systematic and symptom-activated ECG recordings can increase diagnostic yield in such patients. We evaluated the diagnostic accuracy of a simple, leadless, patient-operated ECG device compared with a standard 12-lead ECG. METHODS AND RESULTS: We recorded a standard 12-lead surface ECG and a patient-activated ECG in direct succession in 508 consecutive patients enrolled in four centres. All ECGs were analysed by a single, blinded observer. ECGs were analysable in 505 (99.4%) patients (66% male, age 61 +/- 15 years, and body mass index 27 +/- 4). Analysis of the patient-activated ECG adequately detected a normal ECG (sensitivity 91% and specificity 95%), atrial fibrillation (AF) (sensitivity 99% and specificity 96%), and even T-wave abnormalities (sensitivity 90% and specificity 75%). Diagnostic accuracy for atrioventricular nodal block was moderate (sensitivity 79% and specificity 99%). Continuous parameters correlated well: (r(2) = 0.89 for heart rate, 0.83 for PR interval, 0.78 for QRS duration, and 0.89 for QTc). CONCLUSION: Recordings made by this patient-operated ECG device allow to detect arrhythmias and other ECG changes with high accuracy compared with a standard ECG. It may help to improve accurate diagnosis of transient ECG changes such as paroxysmal AF in palpitations or other unexplained cardiac symptoms.

Stenting of modified Blalock-Taussig shunt in adult with palliated pulmonary atresia and ventricular septal defect: a case report.

BACKGROUND: Adults with complex congenital heart disease palliated with systemic-to-pulmonary artery shunts have become rare and represent a particularly challenging patient group for the cardiologist. One of the complications and causes of severe clinical deterioration during long-term follow-up are progressive obstruction or total occlusion of the shunt. The risk for surgical intervention is frequently high and catheter intervention may be complicated by complex anatomy and shunt calcification. CASE SUMMARY: We report the case of a 47-year-old man with uncorrected (palliated) pulmonary atresia and ventricular septal defect who presented with progressive cyanosis (oxygen saturation 69%) and decreasing exercise capacity. Computed tomography revealed a totally occluded modified left Blalock-Taussig (BT) shunt and a severely stenosed central shunt (Waterston-Cooley) in a patient with confluent but hypoplastic pulmonary arteries and multiple major aortic pulmonary collaterals. Due to a high operative risk, an interventional, percutaneous approach was preferred to re-do surgery. From a radial access the calcified BT shunt could be crossed with a hydrophilic guidewire. Then, a rotational thrombectomy, balloon dilatation, and bare-metal stenting at the proximal and distal anastomoses were performed. Post-interventionally, peripheral oxygen saturation increased from 69% to 82%. Clopidogrel was administered for 1 month after bare-metal stenting. At 1-year follow-up, the BT shunt was still patent on echocardiography and exercise tolerance markedly improved. DISCUSSION: This case highlights the benefit of percutaneous rotational thrombectomy followed by stenting of chronically occluded systemic-to-pulmonary artery shunts for further palliation in adult patients with complex congenital heart disease not suitable for surgical repair.

Impact of Percutaneous Pulmonary Valve Implantation on the Timing of Reintervention for Right Ventricular Outflow Tract Dysfunction.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. Early surgical repair has dramatically improved the outcome of this condition. However, despite the success of contemporary approaches with early complete repair, these are far from being curative and late complications are frequent. The most common complication is right ventricle outflow tract (RVOT) dysfunction, affecting most patients in the form of pulmonary regurgitation, pulmonary stenosis, or both, and can lead to development of symptoms of exercise intolerance, arrhythmias, and sudden cardiac death. Optimal timing of restoration of RVOT functionality in asymptomatic patients with RVOT dysfunction after TOF repair is still a matter of debate. Percutaneous pulmonary valve implantation, introduced almost 2 decades ago, has become a major game-changer in the treatment of RVOT dysfunction. In this article we review the pathophysiology, the current indications, and treatment options for RVOT dysfunction in patients after TOF repair with a focus on the role of percutaneous pulmonary valve implantation in the therapeutic approach to these patients.

Delayed pacemaker requirement after transcatheter aortic valve implantation with a new-generation balloon expandable valve: Should we monitor longer?

OBJECTIVES: To analyze the timing of appearance of conduction abnormalities (CAs) after transcatheter aortic valve implantation (TAVI), to identify predictors of delayed CAs requiring pacemaker (PM) implantation and to provide guidance regarding the duration of telemetry monitoring. BACKGROUND: How long patients remain at risk of development of CAs requiring PM implantation after TAVI and for how long they should be monitored remains unclear but is crucial when considering early discharge. METHODS: Development of CAs was studied in 701 consecutive patients treated with Edwards Sapien 3 valves and monitored with telemetry for 7 days in a single center. After excluding valve-in-valve procedures and patients with previous PM, 606 patients remained for analysis. Predictors of CAs requiring PM and the time of onset of CAs were analyzed. RESULTS: Of 606 patients 76 (12.5%) required a PM after TAVI. CAs requiring PM implantation occurred after 48 h in 22.4% (17 patients) and in 10.5% (8 patients) even after 5 days. Of the patients who developed high grade CAs requiring PM after 48 h, 47.1% had no CAs prior to TAVI, and 23.5% had neither pre-existing CAs nor new-developed CAs within the first 48 h after TAVI. CONCLUSION: After TAVI using a new-generation balloon-expandable valve, delayed development of CAs requiring PM implantation is not uncommon, even after 5 days. More importantly, 23.5% of patients eventually requiring a delayed PM implantation had still no CAs at 48 h after TAVI in this study. These results question the safety of early discharge and support ECG monitoring for a longer time period. The most optimal way to monitor these patients is yet to be determined.