Pulmonary valve replacement in tetralogy of Fallot - who and how?

BACKGROUND AND AIM: Pulmonary regurgitation is the most common complication in repaired tetralogy of Fallot patients. Severe chronic pulmonary regurgitation can be tolerated for decades, but if not treated, it can progress to symptomatic, irreversible right ventricular dilatation and dysfunction. We investigated clinical associations with pulmonary valve replacement among patients with significative pulmonary regurgitation and how interventional developments can change their management. METHODS: All adult patients with repaired tetralogy of Fallot who were followed at an adult CHD Clinic at a single centre from 1980 to 2022 were included on their first outpatient visit. Follow-up was estimated from the time of correction surgery until one of the following events occurred first: pulmonary valve replacement, death, loss to follow-up or conclusion of the study. RESULTS: We included 221 patients (116 males) with a median age of 19 (18-25). At a median age of 33 (10) years old, 114 (51%) patients presented significant pulmonary regurgitation. Among patients with significant pulmonary regurgitation, pulmonary valve replacement was associated with male gender, older age at surgical repair, and longer QRS duration in adulthood. Pulmonary valve replacement was performed in 50 patients, including four transcatheter pulmonary valve implantations, at a median age of 34 (14) years. CONCLUSION: Pulmonary regurgitation affects a large percentage of tetralogy of Fallot adult patients, requiring a long-term clinical and imaging follow-up. Sex, age at surgical repair and longer QRS are associated with the need of PVR among patients with significative pulmonary regurgitation. Clinical practice and current literature support TPVI as the future gold standard intervention.

Treatment of intracardiac thrombi using ultra-slow low-dose thrombolytic therapy: A case report.

A 57-year-old male with previously known severe primary mitral regurgitation was admitted to the intensive care unit (ICU) due to massive venous thromboembolism, associated with right ventricular dysfunction and two large mobile right atrial thrombi. Due to deterioration in his clinical condition despite standard treatment with unfractionated heparin, it was decided to use an ultra-slow low-dose thrombolysis protocol, which consisted of a 24-hour infusion of 24 mg of alteplase at a rate of 1 mg per hour, without initial bolus. The treatment was continued for 48 consecutive hours, with clinical improvement and resolution of the intracardiac thrombi and no complications. One month after ICU admission, successful mitral valve repair surgery was conducted. This case demonstrates that ultra-slow low-dose thrombolysis is a valid bailout treatment option in patients with large intracardiac thrombi refractory to the standard approach.

The world upside down - after 20 years of follow-up of dextro-transposition of the great arteries.

Dextro-transposition of the great arteries (D-TGA) is a congenital heart disease (CHD) classically palliated with atrial switch (ATR-S) and nowadays corrected with arterial switch (ART-S). Our aim was to observe a group of D-TGA patients followed in an adult CHD outpatient clinic. We analyzed a group of D-TGA patients born between 1974 and 2001. Adverse events were defined as a composite of death, stroke, myocardial infarction or coronary revascularization, arrhythmia, and ventricular, baffle, or significative valvular dysfunction. A total of 79 patients were enrolled, 46% of whom were female, with a mean follow-up of 27±6 years after surgery. ATR-S was performed in 54% and ART-S in 46%; the median age at procedure was 13 months and 10 days, respectively. During follow-up, almost all ART-S remained in sinus rhythm versus 64% of ATR-S (p=0.002). The latter group had a higher incidence of arrhythmias (41% versus 3%, p<0.001), mostly atrial flutter or fibrillation; the median time to first arrhythmia was 23 years. Systemic ventricle systolic dysfunction (SVSD) was more frequent in ATR-S (41% versus 0%, p<0.001); the mean time to SVSD was 25 years. In ART-S, the most frequent complication was significant valvular regurgitation (14%). Regarding time-to-event analysis, 80% and 40% of ATR-S maintained adverse events-free after 20 and 30 years, respectively; the time-to-first adverse event was 23 years, and there was no difference compared to ART-S (Log-rank=0.596). ART-S tended to maintain more preserved biventricular function than ATR-S (Log-rank=0.055). After a long term free of adverse events, ATR-S patients experienced more arrhythmias and SVSD. ART-S complications were predominantly anastomosis-related; SVSD or arrhythmias were rare.

Cardiovascular toxicity in breast cancer patients - contributors and role of cardioprotective drugs.

Breast cancer (BC) patients treated with anthracyclines and/or anti-HER2-targeted therapies (AHT) are highly associated with cardiovascular toxicity (CVT). Our objective was to evaluate the risk of CVT secondary to cancer treatment and the role of cardioprotective-drugs (CPD) in BC patients. We collected a retrospective cohort of females with BC treated with chemotherapy and/or AHT from 2017 to 2019. CVT was defined as LVEF<50% or decline ≥10% during follow-up. As CPD, we considered renin-angiotensin-aldosterone-system inhibitors and beta-blockers. A subgroup analysis of the AHT patients was also performed. A total of 203 women were enrolled. The majority had high or very-high CVT risk score and normal cardiac function at presentation. As for CPD, 35.5% were medicated pre-chemotherapy. All patients were submitted to chemotherapy; AHT were applied to 41.7%. During a 16 months follow-up, 8.5% developed CVT. There was a significant decrease of GLS and LVEF at 12-months (decrease of 1.1% and 2.2%, p<0.001). AHT and combined therapy were significantly associated with CVT. In the AHT sub-group analysis (n=85), 15.7% developed CVT. Patients previously medicated with CPD had a significative lower incidence of CVT (2.9% vs 25.0%, p=0.006). Patients already on CPD presented a higher LVEF at 6-months follow-up (62.5% vs 59.2%, p=0.017). Patients submitted to AHT and anthracycline therapy had higher risk of developing CVT. In the AHT sub-group, pre-treatment with CPD was significantly associated with a lower prevalence of CVT. These results highlight the importance of cardio-oncology evaluation and strengthen the value of primary prevention.

An Unexpected Cause of Acute Abdomen Late After Heart Transplantation-A Case Report.

Cytomegalovirus (CMV) infection is a frequent complication after a solid organ transplant, and in 86% of the cases, CMV disease occurred during the first 6 months after transplantation. Invasive CMV infections may be present as ulcerative infections of the upper gastrointestinal tract with esophagitis, gastritis, and ulcerations of the duodenum and the small bowel; however, CMV infections of the pancreatobiliary system, especially papillitis, are rarely observed. We present a case report of a man who underwent a heart transplant 6 years before, with a clinical picture of duodenitis and a simultaneous pseudotumor of major duodenal papilla who developed signs of acute abdomen caused by gastrointestinal CMV infection, successfully treated with medical therapy with valganciclovir. There is an urgent need for developments in CMV and solid organ transplantation to stratify the risk of late-onset CMV disease.

First description of Portuguese patients with cardiac amyloidosis and p.Val142Ile: more evidence of an "African variant" in Caucasians.

Objectives. Hereditary transthyretin amyloidosis caused by the (ATTRv) p. Val142Ile variant is a common cause of cardiac amyloidosis among Western African countries and Afro-Americans populations. However, in recent years, Caucasian patients have been identified in greater numbers, raising the question of whether this variant has been undeappreciated in this population. We now have new cases of cardiac amyloidosis caused by the p.Val142Ile from a center in northern Portugal. In addition, we reviewed and discussed the published data concerning p.Val142Ile in Caucasians. Design. Patients diagnosed with cardiac amyloidosis underwent genetic testing using TTR gene sequencing and their relatives were recommended for genetic counsellingand testing if a pathogenic TTR variant was found. In our center, we reviewed the clinical data of patients who had the p.Val142Ile variant. A review of published cases of p.Val142Ile in Caucasians was also performed, to which our data was compared. Results. We found three ATTRv patients with the p.Val142Ile variant (one homozygotic), all Caucasian males with a median age at diagnosis of 69 years old. All of them had heart failure and arrhythmias. During the follow-up period, two patients died. There were 47 unrelated unrelated Caucasian cases of ATTRv p.Val142Ile variant reported worldwide until May 2022. Conclusions. Our findings add to the mounting evidence that the global prevalence of p.Val142Ile is likely understated. This highlights the importance of the systematic screening of the TTR gene in amyloidosis and phenocopies, as well as larger epidemiologic studies to determine the true ATTRv p.Val142Ile prevalence in non-African communities.

Wernicke encephalopathy and beriberi disease presenting as STEMI-equivalent.

Thiamine deficiency is commonly associated with malnutrition, alcoholism and bariatric surgery. Thiamine deficiency can manifest in different ways, especially in developing countries: as peripheric neuropathy, as Wernicke encephalopathy or as beriberi disease. The authors present the case of a 72-year-old male, with a hiatal hernia that led to thiamine deficiency due to malnutrition. The initial clinical manifestation was an ST-elevation myocardial infarct equivalent, an ECG with a shark-fin pattern that evolved to a Wellens type B pattern. The patient evolved with severe altered mental status. A Wernicke encephalopathy diagnosis was confirmed by MRI; the patient was medicated with high-dose thiamine, with quick recovery, both neurologic and cardiac. The clinical history and response to treatment confirm the diagnosis of Wernicke encephalopathy and beriberi disease.

A new era in patent foramen ovale closure - a percutaneous suture-based 'deviceless' technique (NobleStitch®): Experience of a Portuguese center.

INTRODUCTION: In patients with cryptogenic stroke, one of the most frequently found abnormalities is patent foramen ovale (PFO). Percutaneous 'deviceless' systems based on surgical suture-mediated PFO closure have recently been introduced and show a favorable efficacy and safety profile with clear advantages. OBJECTIVES: To present procedural details of the technique and baseline characteristics of patients who underwent the procedure in our center. METHODS: A single-center prospective observational registry was established between February 2020 and February 2021, to assess the safety, efficacy and possible advantages of a novel percutaneous PFO closure system (NobleStitch® EL). Patient and PFO characteristics as well as technical features were collected for analysis. RESULTS: Twenty-three patients were considered suitable for this technique after transesophageal echocardiography. Their mean age was 51 years and 69.5% were women. Most patients (91.3%) had a history of cryptogenic stroke. PFO closure with the NobleStitch® system was successfully performed in all patients. All procedures were performed under local anesthesia and fluoroscopic monitoring. The mean duration of the procedure was 52 min and median contrast dose used was 187 ml. Median radiation dose absorbed per patient was 61.5 Gy cm2. All patients were discharged asymptomatic 24 hours after the procedure with no peri- or postprocedural complications recorded. CONCLUSION: Suture-mediated PFO closure represents a valid and safe alternative to traditional umbrella-like devices, and is feasible in the majority of PFO anatomies. Follow-up information, results of larger series and clinical trials may possibly validate this technique as the first choice for PFO closure.

Coronary artery fistulas: A 12-year single-center experience.

INTRODUCTION AND OBJECTIVES: Coronary artery fistulas (CAFs) are rare abnormalities, often detected incidentally during invasive coronary angiography (ICA). While most are clinically silent, they can cause significant morbidity. We aimed to investigate the clinical, angiographic and management features of CAFs in a population undergoing ICA. METHODS: We retrospectively reviewed the data of all ICAs conducted in our department between May 2008 and January 2020 and selected those with CAFs. Clinical, angiographic, therapeutic and follow-up data were obtained from medical records. RESULTS: A total of 55 patients with CAFs (35 male, median age 64 years) were identified among 32 174 ICAs. The majority (n=37) had a single fistula. CAFs arose most frequently from the left anterior descending artery (LAD), followed by the right coronary and left circumflex coronary arteries. The most frequent drainage site was the pulmonary artery. Fourteen patients had fistulas originating from both left and right coronary systems. Seven had concomitant congenital cardiovascular disorders. The majority (n=40) were incidental findings. Chest pain was the most common symptom attributable to CAFs and heart murmur the most frequent sign. Conservative management was the main approach (n=40). Eight patients underwent transcatheter closure and seven underwent surgical ligation (six of those during surgery for another heart condition), with no periprocedural mortality. CONCLUSIONS: In our series, the prevalence of CAFs was 0.2%. The majority originated from the LAD and the pulmonary artery was the main drainage site. In patients undergoing intervention, both percutaneous and surgical techniques were safe and effective.

Acute Myocarditis: A New Manifestation of Monkeypox Infection?

A 31-year-old male patient with confirmed monkeypox infection developed acute myocarditis days after the eruption of skin lesions. Cardiac magnetic resonance study confirmed myocardial inflammation. The patient was treated with supportive care and had full clinical recovery. This case highlights cardiac involvement as a potential complication associated with monkeypox. (Level of Difficulty: Intermediate.).

Dependência de Pacing a Longo-Prazo e Preditores de Implante de Pacemaker após Implante Percutâneo de Prótese Valvular Aórtica - 1 Ano de Seguimento / Long-Term Ventricular Pacing Dependency and Pacemaker Implantation Predictors after Transcatheter Aortic Valve Replacement - A 1-Year Follow-Up

Resumo Fundamento Os distúrbios de condução (DC) são a complicação mais frequente após a substituição da válvula aórtica transcateter (TAVR) e ainda não há consenso sobre seu tratamento. Objetivo Avaliar novos DC e implante de marca-passo definitivo (MPD) após a TAVR e avaliar a porcentagem de estimulação ventricular (EV) até 1 ano de acompanhamento. Métodos Pacientes submetidos a TAVR de outubro de 2014 a novembro de 2019 foram cadastrados; pacientes com MPD anterior foram excluídos. Dados clínicos, do procedimento, do ECG e do MPD foram coletados até 1 ano após o implante. O nível de significância adotado para a análise estatística foi 0,05%. Resultados Um total de 340 indivíduos foram submetidos a TAVR. O DC mais comum foi bloqueio de ramo esquerdo novo (BRE; 32,2%), sendo que 56% destes foram resolvidos após 6 meses. O bloqueio do ramo direito (BRD) foi o maior fator de risco para bloqueio atrioventricular avançado (BAV) [RC=8,46; p<0,001] e implante de MPD [RC=5,18; p<0,001], seguido de BAV de baixo grau prévio [RC=2,25; p=0,016 para implante de MPD]. Em relação às características do procedimento, válvulas de gerações mais recentes e procedimentos de válvula-em-válvula foram associados a menos DC. No total, 18,5% dos pacientes tiveram MPD implantado após a TAVR. Na primeira avaliação do MPD, pacientes com BAV avançado tinham uma porcentagem mediana de EV de 80%, e, após um ano, de 83%. Em relação aos pacientes com BRE e BAV de baixo grau, a EV mediana foi mais baixa (6% na primeira avaliação, p=0,036; 2% após um ano, p = 0,065). Conclusão O BRE foi o DC mais frequente após a TAVR, com mais da metade dos casos se resolvendo nos primeiros 6 meses. O BRD foi o principal fator de risco para BAV avançado e implante de MPD. O BAV avançado foi associado a uma porcentagem mais alta de EV no acompanhamento de 1 ano.

Abstract Background Conduction disturbances (CD) are the most frequent complication after transcatheter aortic valve replacement (TAVR), and there continues to be a lack of consensus on their management. Objective To assess new CD and permanent pacemaker (PPM) implantation after TAVR and to evaluate the ventricular pacing percentage (VP) up to 1 year of follow-up. Methods Patients who underwent TAVR from October 2014 to November 2019 were enrolled; patients with previous PPM were excluded. Clinical, procedure, ECG, and PPM data were collected up to 1 year after implantation. The significance level adopted in the statistical analysis was 0.05. Results A total of 340 patients underwent TAVR. The most frequent CD was the new left bundle branch block (LBBB; 32.2%), which 56% resolved after 6 months. Right bundle branch block (RBBB) was the biggest risk factor for advanced atrioventricular block (AVB) [OR=8.46; p<0.001] and PPM implantation [OR=5.18, p<0.001], followed by previous low-grade AVB [OR=2.25; p=0.016 for PPM implantation]. Regarding procedure characteristics, newer generation valves and valve-in-valve procedures were associated with fewer CDs. Overall, 18.5% of patients had a PPM implanted post-TAVR. At first PPM evaluation, patients with advanced AVB had a median percentage of VP of 80% and 83% at one year. Regarding patients with LBBB plus low-grade AVB, median VP was lower (6% at first assessment, p=0.036; 2% at one year, p = 0.065). Conclusion LBBB was the most frequent CD after TAVR, with more than half being resolved in the first six months. RBBB was the major risk factor for advanced AVB and PPM implantation. Advanced AVB was associated with a higher percentage of VP at 1 year of follow-up.

Long-Term Ventricular Pacing Dependency and Pacemaker Implantation Predictors after Transcatheter Aortic Valve Replacement - A 1-Year Follow-Up. / Dependência de Pacing a Longo-Prazo e Preditores de Implante de Pacemaker após Implante Percutâneo de Prótese Valvular Aórtica ­ 1 Ano de Seguimento.

BACKGROUND: Conduction disturbances (CD) are the most frequent complication after transcatheter aortic valve replacement (TAVR), and there continues to be a lack of consensus on their management. OBJECTIVE: To assess new CD and permanent pacemaker (PPM) implantation after TAVR and to evaluate the ventricular pacing percentage (VP) up to 1 year of follow-up. METHODS: Patients who underwent TAVR from October 2014 to November 2019 were enrolled; patients with previous PPM were excluded. Clinical, procedure, ECG, and PPM data were collected up to 1 year after implantation. The significance level adopted in the statistical analysis was 0.05. RESULTS: A total of 340 patients underwent TAVR. The most frequent CD was the new left bundle branch block (LBBB; 32.2%), which 56% resolved after 6 months. Right bundle branch block (RBBB) was the biggest risk factor for advanced atrioventricular block (AVB) [OR=8.46; p<0.001] and PPM implantation [OR=5.18, p<0.001], followed by previous low-grade AVB [OR=2.25; p=0.016 for PPM implantation]. Regarding procedure characteristics, newer generation valves and valve-in-valve procedures were associated with fewer CDs. Overall, 18.5% of patients had a PPM implanted post-TAVR. At first PPM evaluation, patients with advanced AVB had a median percentage of VP of 80% and 83% at one year. Regarding patients with LBBB plus low-grade AVB, median VP was lower (6% at first assessment, p=0.036; 2% at one year, p = 0.065). CONCLUSION: LBBB was the most frequent CD after TAVR, with more than half being resolved in the first six months. RBBB was the major risk factor for advanced AVB and PPM implantation. Advanced AVB was associated with a higher percentage of VP at 1 year of follow-up.

FUNDAMENTO: Os distúrbios de condução (DC) são a complicação mais frequente após a substituição da válvula aórtica transcateter (TAVR) e ainda não há consenso sobre seu tratamento. OBJETIVO: Avaliar novos DC e implante de marca-passo definitivo (MPD) após a TAVR e avaliar a porcentagem de estimulação ventricular (EV) até 1 ano de acompanhamento. MÉTODOS: Pacientes submetidos a TAVR de outubro de 2014 a novembro de 2019 foram cadastrados; pacientes com MPD anterior foram excluídos. Dados clínicos, do procedimento, do ECG e do MPD foram coletados até 1 ano após o implante. O nível de significância adotado para a análise estatística foi 0,05%. RESULTADOS: Um total de 340 indivíduos foram submetidos a TAVR. O DC mais comum foi bloqueio de ramo esquerdo novo (BRE; 32,2%), sendo que 56% destes foram resolvidos após 6 meses. O bloqueio do ramo direito (BRD) foi o maior fator de risco para bloqueio atrioventricular avançado (BAV) [RC=8,46; p<0,001] e implante de MPD [RC=5,18; p<0,001], seguido de BAV de baixo grau prévio [RC=2,25; p=0,016 para implante de MPD]. Em relação às características do procedimento, válvulas de gerações mais recentes e procedimentos de válvula-em-válvula foram associados a menos DC. No total, 18,5% dos pacientes tiveram MPD implantado após a TAVR. Na primeira avaliação do MPD, pacientes com BAV avançado tinham uma porcentagem mediana de EV de 80%, e, após um ano, de 83%. Em relação aos pacientes com BRE e BAV de baixo grau, a EV mediana foi mais baixa (6% na primeira avaliação, p=0,036; 2% após um ano, p = 0,065). CONCLUSÃO: O BRE foi o DC mais frequente após a TAVR, com mais da metade dos casos se resolvendo nos primeiros 6 meses. O BRD foi o principal fator de risco para BAV avançado e implante de MPD. O BAV avançado foi associado a uma porcentagem mais alta de EV no acompanhamento de 1 ano.

Evaluation of Usability, Perception of Usefulness, and Efficiency of an Application in Interpreting Imaging Examinations and Supporting Decision-Making in Orthopedics.

Background: Medical smartphone applications have rendered positive results in daily practice and also serve as potentially valuable educational tools. In orthopedics, radiograph analysis and angle measurements comprise an essential mechanism in the diagnosis, treatment, planning, and evaluation of the results in orthopedic surgery. Purpose: Thus, the present study aimed to evaluate an application for mobile devices for orthopedic doctors and orthopedic residents. The application proposes the analysis and interpretation of radiographic imaging examinations through assessments and angle measurements. In this survey, we analyzed the parameter usability and usefulness of a mobile application for the iOS system, in addition to conducting its validation. Patients and Methods: The application was tested by 26 volunteers: 13 orthopedists and 13 orthopedic residents. Results: After applying the System Utility Score (SUS) questionnaire, which assesses the usability of an application, a score of 84.5 was obtained, a value that represents an A+ rating on the Sauro and Lewis scale. There was no statistically significant difference when comparing the SUS between the orthopedic residents and orthopedists. According to the usefulness perception analysis, 90% of the residents responded positively to the questions, while among orthopedists, the percentage was 75%, denoting a statistically significant difference (p = 0.002). Subsequently, the application underwent validation to assess whether the calculated angle measurements were reliable when compared with those obtained using the goniometer. No significant differences were observed when comparing the angles measured by the two evaluated methods. Conclusion/Clinical Relevance: In conclusion, the developed application can reliably contribute to the measurement of angles in radiographs, assisting in therapeutic decision-making, and may be used as an instrument to promote the teaching and learning processes in orthopedics.

Left ventricular pacing with a temporary pacemaker: Case report.

We report a case of temporary pacemaker lead malposition in the left ventricle crossing the interventricular septum (IVS). The majority of described cases occur due to a patent foramen ovale and are frequently incidental findings. A course across the IVS is rarely found and this complication with temporary leads is not even reported in the literature. This very rare location entails a risk of dangerous complications associated with left-to-right flow after lead removal. Echocardiography was an essential tool to diagnose the lead's course inside the heart and enabled secure removal of the lead with cardiac surgery backup.