Complete heart block caused by sinus of valsalva pseudoaneurysm-A rare case.

Acquired pseudoaneurysms of the aortic root involving the sinus of Valsalva (SOV) are rare and serious complications arising from trauma, infection, or following cardiac surgery or intervention. Complete heart block (CHB) is an atypical presentation of SOV pseudoaneurysm due to either direct compression effects or involvement of the main conducting system by blood and inflammatory cell infiltration. Herein, we describe a rare case of a patient who presented with CHB caused by an SOV pseudoaneurysm following polytrauma and was treated with surgical closure of pseudoaneurysm followed by implantation of a permanent pacemaker to treat the persistent CHB.

Systemic Diseases and Heart Block.

Systemic diseases can cause heart block owing to the involvement of the myocardium and thereby the conduction system. Younger patients (<60) with heart block should be evaluated for an underlying systemic disease. These disorders are classified into infiltrative, rheumatologic, endocrine, and hereditary neuromuscular degenerative diseases. Cardiac amyloidosis owing to amyloid fibrils and cardiac sarcoidosis owing to noncaseating granulomas can infiltrate the conduction system leading to heart block. Accelerated atherosclerosis, vasculitis, myocarditis, and interstitial inflammation contribute to heart block in rheumatologic disorders. Myotonic, Becker, and Duchenne muscular dystrophies are neuromuscular diseases involving the myocardium skeletal muscles and can cause heart block.

Outcomes of Valvular Endocarditis in Patients With and Without Pericardial Effusion: A National Inpatient Sample Study.

Over the last decade, hospitalizations for infective endocarditis (IE) have been steadily increasing, leading to a significant healthcare burden. Pericardial effusion (PCE) has been identified as a serious complication of IE, yet no significant association with mortality has been established. Our study aims to further analyze and understand the significance of PCE in patients with IE. We performed a retrospective analysis using the national inpatient sample database to identify all the hospital admissions with IE using ICD 10 codes and stratified them into 2 groups based on the presence of PCE. The outcomes of interest were inhospital mortality, inhospital complications, need for cardiac surgery, and length of stay. From 2015 Q4-2019, a total of 76,260 hospitalizations were included (weighted: 381,300), of which 2.7% included a PCE diagnosis. Hospitalizations with a PCE diagnosis included patients that were younger (51 vs 61, P < 0.001), as well as slightly more males (58.0% vs 55.2%, P = 0.011), and black patients (16.9% vs 12.9%, P < 0.001). PCE was associated with higher in-hospital death (12.7% vs 9.0%, P < 0.001), longer lengths of stay (12 days vs 7 days, P < 0.001), higher rates of cardiac surgery (22.4% vs 7.3%, P < 0.001). The rates of heart failure, heart block, renal failure, cardiogenic shock, and embolic stroke were higher on PCE group. We found that presence of PCE is associated with higher inhospital mortality, longer length of stay, and greater utilization of cardiac surgery, as well as presence of heart failure, heart block, cardiogenic shock, and embolic stroke.

Complete Heart Block After Low Dose Octreotide Infusion for Esophageal Variceal Bleeding.

Cirrhosis and chronic liver disease cause a myriad of systemic health problems mostly caused by the presence of portal hypertension. Esophageal varices are one result of portal hypertension. They can rupture and bleed, which can be catastrophic in already coagulopathic liver failure patients. We present a patient who presented with decompensated liver failure for transplant. He developed a severe and refractory gastrointestinal bleed and was put on an octreotide infusion to increase splanchnic flow and decrease portal pressures. He subsequently developed complete heart block. Understanding the mechanisms of octreotide is imperative due to its frequent use in medically complex patients.

Aseptic presentation of interventricular septal abscess with progressive heart block: a case report.

BACKGROUND: Infective endocarditis can progress to an intracardiac abscess in 20% to 30% of cases, with interventricular septal abscess (IVSA) being one of the rare complications usually presenting with sepsis. We present a case of IVSA presenting with a new-onset second-degree heart block, which rapidly progressed to a complete heart block. CASE PRESENTATION: A 80-year-old Caucasian female with a past medical history of hypertension and hyperlipidemia presented with exertional chest pain, lightheadedness, and shortness of breath with telemetry and electrocardiogram revealing persistent Mobitz type II second degree atrioventricular block. The rest of the vitals were normal. As she was being planned for a pacemaker placement, she spiked a temperature of 103F. Blood cultures grew methicillin-sensitive Staphylococcus aureus, and appropriate antibiotics were initiated. Transthoracic echocardiogram was grossly normal. However, transesophageal echocardiogram revealed a heterogeneous extension of an echodensity from the aortic root, along the aorto-mitral cushion and into the interventricular septum, indicating an interventricular septal abscess. Her course was complicated by altered mental status, with computed tomography of the brain revealing hypodense regions in the left lentiform nucleus and anterior caudate nucleus representing acute/subacute stroke. Surgery was deferred as she was deemed a poor candidate. She succumbed to her illness on day 6 of hospitalization. CONCLUSION: Intracardiac abscesses should be considered a possible initial differential in patients with progressive heart block despite aseptic presentation and no risk factors.

Reporting complete heart block in a patient with polyarteritis nodosa after COVID-19 vaccination.

Complete heart block (CHB) is a serious health condition, and polyarteritis nodosa (PAN) is an important autoimmune disease. In the COVID-19 pandemy, several vaccines were developed for the COVID-19 disease that shown several side effects, and some of these complications are still unknown. This is the first report of CHB in a patient with history of PAN after COVID-19 vaccination. A 68-year-old man with a history of PAN referred to our hospital, complaining of presyncope episodes and dizziness after receiving a COVID-19 vaccine. Physical examination, laboratory tests, and transthoracic echocardiography were normal. In his electrocardiogram, a narrow QRS complex, AV dissociation, and junctional escape rhythm were seen. Coronary angiography showed a mild coronary artery disease. The patient, suffering from PAN for years, was hypothesized due to CHB a few days after COVID-19 vaccination. This case report suggests that COVID-19 vaccines may interrupt the conduction system of the heart and the fact that underlying PAN may predispose to CHB following COVID-19 vaccination. Further studies are needed to accurately assess a possible association between PAN, CHB, and COVID-19 vaccines.

Cardiac Manifestations of Lyme Disease.

Lyme carditis is an uncommon manifestation of Lyme disease. Most cases present with heart block of varying degrees, but the spectrum of disease includes other transient arrhythmias and structural manifestations, such as myopericarditis or cardiomyopathy. Antibiotics hasten the resolution of Lyme carditis, and cardiac pacing can be an adjunctive therapy. Outcomes are generally good, but there are rare fatalities associated with Lyme carditis. The latter underscores the continued need for improved modes of prevention of Lyme disease and the importance of its early recognition and treatment.

Impact of Hospital Volume on Outcomes of Septal Myectomy for Hypertrophic Cardiomyopathy.

BACKGROUND: Left ventricular outflow tract obstruction is common among symptomatic patients with hypertrophic cardiomyopathy, yet septal reduction by surgical myectomy (septal myectomy [SM]) is performed infrequently in many centers. This study examined the possible relationship between institutional case volume and early outcomes of SM. METHODS: The Society of Thoracic Surgeons Adult Cardiac Surgery Database was queried for patients with hypertrophic cardiomyopathy who underwent SM from January 2012 to December 2019. The study defined center case volume categories as <1, 1 to 4.99, 5 to 9.99, and ≥10 cases performed on average per year. RESULTS: The study population included 5935 patients at 481 centers with 933 surgeons. The range of average center volume was <1 to 138 cases per year. Overall early mortality was 2.6%, ventricular septal defect (VSD) occurred in 1.9%, and complete heart block occurred in 9.0%. Concomitant mitral valve (MV) repair was performed in 28.7%, and MV replacement was performed in 17.1%. In multivariable analysis, the lowest annual case volume (average <1 case/y) was consistently associated with greater early mortality (odds ratio [OR], 5.4; CI, 3.0-9.9; P < .001), greater risk of VSD (OR, 9.3; CI ,4.2-20.4; P < .001), increased incidence of complete heart block (OR, 2.0; CI, 1.5-2.7; P < .001), and a higher likelihood of MV replacement (OR, 9.4; CI, 7.5-11.8; P < .001). CONCLUSIONS: Volume of SM cases varies widely among institutions reporting to the Society of Thoracic Surgeons Adult Cardiac Surgery Database. There appears to be an important association between surgical experience, as reflected by institutional case volume, and early outcomes, including mortality, as well as the occurrence of VSD, heart block, and MV replacement.

Association of early electrical changes with cardiovascular outcomes in immune checkpoint inhibitor myocarditis.

BACKGROUND: Immune-checkpoint inhibitor-associated myocarditis (ICI-myocarditis) often presents with arrhythmias, but the prognostic value of early electrocardiogram findings is unclear. Although ICI-myocarditis and acute cellular rejection (ACR) following cardiac transplantation use similar treatment strategies, differences in arrhythmia burden are unknown. OBJECTIVE: To evaluate the association of electrocardiogram findings in ICI-myocarditis with myocarditis-related mortality and life-threatening arrhythmia. METHODS: A total of 125 cases of ICI-myocarditis were identified retrospectively across 49 hospitals worldwide; 50 cases of grade 2R or 3R ACR were included as comparators. Two cardiologists blinded to clinical data interpreted electrocardiograms. Associations between electrocardiogram features, myocarditis-related mortality and the composite of myocarditis-related mortality and life-threatening arrhythmias were examined. Adjusted hazard ratios (aHRs) were calculated. RESULTS: The cohort had 78 (62.4%) men; median (interquartile range) age was 67 (58-76) years. At 30 days, myocarditis-related mortality was 20/124 (16.1%), and 28/124 (22.6%) met the composite endpoint. Patients who developed complete heart block (aHR by subdistribution hazards model [aHR(sh)] 3.29, 95% confidence interval [CI] 1.24-8.68; P=0.02) or life-threatening cardiac arrhythmias (aHR(sh) 6.82, 95% CI: 2.87-16.21; P<0.001) had a higher risk of myocarditis-related mortality. Pathological Q waves (aHR(sh) 3.40, 95% CI: 1.38-8.33; P=0.008), low QRS voltage (aHR(sh) 6.05, 95% CI: 2.10-17.39; P<0.001) and Sokolow-Lyon index (aHR(sh)/mV 0.54, 95% CI: 0.30-0.97; P=0.04) on admission electrocardiogram were also associated with increased risk of myocarditis-related mortality. These associations were mirrored in the composite outcome analysis. Compared with ACR, ICI-myocarditis had a higher incidence of life-threatening cardiac arrhythmias (15/125 [12.0%] vs 1/50 [2%]; P=0.04) and third-degree heart block (19/125 [15.2%] vs 0/50 [0%]; P=0.004). CONCLUSIONS: Electrocardiograms in ICI-myocarditis with ventricular tachycardias, heart block, low-voltage and pathological Q waves were associated with myocarditis-related mortality and life-threating arrhythmia. Arrhythmia burden in ICI-myocarditis exceeds that of ACR after heart transplant.

Management of Complete Heart Block in a Pregnant Woman with Systemic Lupus Erythematosus-Associated Complications: Treatment Considerations and Pitfalls.

We present a case of a pregnant woman with systemic lupus erythematosus (SLE) who was diagnosed with asymptomatic complete heart block (CHB) during pregnancy. To evaluate possible risks and benefits of pacemaker (PM) implantation, a multidisciplinary counselling board was held. Its recommendation was to perform PM implantation to prevent intra-uterine growth restriction from insufficient cardiac output using a fluoroscopic protective shield. The procedure was performed without complications and established permanent pacing on onwards ECG examinations. The patient subsequently gave birth to a healthy newborn. After a retrospective clinical case evaluation and review of relevant literature, a presumptive association between CHB and the primary diagnosis was proposed. Above that, pregnant women with SLE who develop hypertension are commonly treated with methyldopa, which may cause conduction abnormalities. Clinical recommendations for young female patients expecting pregnancy are lacking in this area. Careful diagnostic and treatment approaches should be used in the management of possible SLE-related complications in women of child-bearing age, focusing on preventable events.

Cohort study of congenital complete heart block among preterm neonates: a single-center experience over a 15-year period.

Congenital complete heart block (CCHB) is a very rare condition, with high risk of mortality. Prematurity is associated with immaturity of the cardiovascular system. Morbidity related to CCHB and prematurity has never been described. We describe a tertiary perinatal center experience over a 15-year period on CCHB management and complications in preterm infants. This is a single-center observational cohort study. All neonates admitted to neonatal intensive care unit with a diagnosis of isolated CCHB between January 2006 and January 2021 were identified. All preterm neonates (< 37 weeks) were compared with a control cohort of term neonates (≥ 37 weeks). Antenatal data, complications of prematurity, medical, and surgical management of CCHB were recorded. Twenty-four neonates with isolated CCHB (16 preterm and 8 term) were born during the study period, including 5 very preterm (< 32 weeks) and 11 preterm (32 to 37 weeks). All very preterm were born via emergency caesarian section without antenatal steroid administration. They had multiple severe morbidities including chronic lung disease, necrotizing enterocolitis, grades 3-4 intraventricular hemorrhage, cystic periventricular leukomalacia, and longer periods of mechanical and non-invasive ventilatory support than preterm. Thirteen out of sixteen preterm infants had permanent pacemakers inserted, compared to 1/8 for term newborns. All babies born before 35-week gestation were either paced or died.Conclusion: Premature neonates with CCHB have high risk of mortality and morbidity especially if undiagnosed and born by unnecessary emergency caesarian section without antenatal steroids. Prematurity below 35 weeks may be associated with death or pacemaker insertion. This supports better antenatal screening to avoid induced prematurity. What is Known: • Congenital complete heart block is a very rare condition associated with high morbidity and mortality. • Antenatal risk factors for poor outcome include fetal hydrops, low ventricular rate (HR <55 beats per minute), and congenital heart defect. What is New: • Infants born <32 weeks with CCHB had no antenatal steroid administration, and sustained high burden of morbidity (chronic lung disease, intraventricular hemorrhage, and cystic periventricular leukomalacia). • Birth <35 weeks is strongly associated with requiring pacing prior to discharge or death.

Case Report Detailing an Inferior Myocardial Infarction, Third-Degree Heart Block, and Cardiogenic Shock.

An infarction in the right coronary artery affects the inferior wall of the heart and can also cause impedance to the cardiac conduction system. The right coronary artery perfuses the sinoatrial and atrioventricular nodes, and a loss of blood flow contributes to a breakdown in the communication system within the heart, causing associated bradycardias, heart blocks, and arrhythmias. This case report details the prehospital and emergency care of a middle-aged man who experienced an inferior myocardial infarction, concomitant third-degree heart block, and subsequent cardiogenic shock, with successful revascularization. This case is informative for emergency clinicians to review symptoms of acute coronary syndrome, rapid lifesaving diagnostics and intervention, and the unique treatment and monitoring considerations associated with right ventricular involvement and third-degree heart block.

Pulmonary hypertension associated with congenital heart block and neonatal lupus syndrome: A series of four cases.

OBJECTIVE: Neonatal lupus syndrome has multisystemic manifestations among which pulmonary involvement has been rarely reported. We describe the clinical presentation, management, and outcome of a series of four neonates who developed reversible pulmonary hypertension associated with auto-immune congenital complete heart block. METHOD: Data from the French registry of neonatal lupus syndrome were retrospectively reviewed. RESULTS: Between 2000 and March 2020, 231 children were included in the French registry, four/73 followed in our institution developed pulmonary hypertension. Diagnosis was suspected on transthoracic echocardiography at a median age of 42 days [range 10-58], and confirmed by right heart catheterization in all; 2 of them where paced at time of diagnosis and 2 were not. All had some degree of hypoxemia and respiratory distress. Hypoxemia was always reversible under O2 et NO. Lung CT demonstrated ground glass anomalies in all. One patient had a lung biopsy consistent with pulmonary hypertension secondary to lung disease. Management included immunosuppressive therapy in 3 associated with sildenafil in 2. Pulmonary hypertension resolved in all at a median age of 4 weeks [range 3-6] after treatment initiation and after one year for the one child who did not receive specific treatment. CONCLUSION: Clinical, hemodynamical, imaging and histological findings advocate for pulmonary hypertension associated with respiratory disease as a rare manifestation of neonatal lupus syndrome.

Congenital complete heart block in the setting of severe pre-eclampsia requiring urgent cesarean section.

Congenital complete heart block is a rare phenomenon that may be discovered during pregnancy in patients who were previously asymptomatic. Peripartum management of these patients mandates a multidisciplinary approach with careful planning regarding indications for pacing, appropriate anesthetic technique, and contingency planning. Approaches to anesthetic management for congenital complete heart block have been described, but management in association with severe pre-eclampsia has not been reported. We describe the anesthetic management of a parturient with complete heart block who presented with severe pre-eclampsia requiring urgent cesarean section.

Cardiac Emerinopathy: A Nonsyndromic Nuclear Envelopathy With Increased Risk of Thromboembolic Stroke Due to Progressive Atrial Standstill and Left Ventricular Noncompaction.

BACKGROUND: Mutations in the nuclear envelope genes encoding LMNA and EMD are responsible for Emery-Dreifuss muscular dystrophy. However, LMNA mutations often manifest dilated cardiomyopathy with conduction disturbance without obvious skeletal myopathic complications. On the contrary, the phenotypic spectrums of EMD mutations are less clear. Our aims were to determine the prevalence of nonsyndromic forms of emerinopathy, which may underlie genetically undefined isolated cardiac conduction disturbance, and the etiology of thromboembolic complications associated with EMD mutations. METHODS: Targeted exon sequencing was performed in 87 probands with familial sick sinus syndrome (n=36) and a progressive cardiac conduction defect (n=51). RESULTS: We identified 3 X-linked recessive EMD mutations (start-loss, splicing, missense) in families with cardiac conduction disease. All 3 probands shared a common clinical phenotype of progressive atrial arrhythmias that ultimately resulted in atrial standstill associated with left ventricular noncompaction (LVNC), but they lacked early contractures and progressive muscle wasting and weakness characteristic of Emery-Dreifuss muscular dystrophy. Because the association of LVNC with EMD has never been reported, we further genetically screened 102 LVNC patients and found a frameshift EMD mutation in a boy with progressive atrial standstill and LVNC without complications of muscular dystrophy. All 6 male EMD mutation carriers of 4 families underwent pacemaker or defibrillator implantation, whereas 2 female carriers were asymptomatic. Notably, a strong family history of stroke observed in these families was probably due to the increased risk of thromboembolism attributable to both atrial standstill and LVNC. CONCLUSIONS: Cardiac emerinopathy is a novel nonsyndromic X-linked progressive atrial standstill associated with LVNC and increased risk of thromboembolism.