Prevalence of neonatal lupus in a small group of antibody-carrying mothers and frequency of complete atrioventricular block.

BACKGROUND: Neonatal lupus (NL) is extremely rare and is caused by the transplacental passage of maternal IgG autoantibodies against Ro, La, and/or RNP proteins into the fetal circulation, which can cause congenital complete atrioventricular block (CCAB), permanent skin lesions, and liver involvement. OBJECTIVE: To know the prevalence of NL in patients with CCAB and the clinical course in long-term follow-up. METHODS: From January 1992 to December 2017, patients with CCAB were included. The presence of anti-SSA/Ro and anti-SSB/La antinuclear antibodies in maternal serum confirmed NL. RESULTS: Eight patients were included with a follow-up of 10 ± 6 years; NL was concluded in 62.5%; two were male. One of them was diagnosed in utero, two at birth, and a pacemaker was implanted in them, one at 12 years of age and another at 15. The other two cases were diagnosed at 18 and 26 years of age, and permanent pacemakers were implanted 8 and 5 years later, respectively. In one case, a definitive pacemaker was not implanted in a newborn with only 1 year of follow-up. At delivery, 60% of the mothers were free of rheumatic disease, and altogether, they all had 19 children; none of them presented NL manifestations. CONCLUSIONS: CCAB is rare and frequently associated with a maternal autoimmune disease, practically all of them will require a definitive pacemaker at some point in their lives.

ANTECEDENTES: El lupus neonatal (LN) es extremadamente raro y es ocasionado por el paso transplacentario de auto-anticuerpos maternos IgG contra las proteínas Ro, La y/o RNP a la circulación fetal que puede ocasionar bloqueo aurículo-ventricular completo congénito (BAVCC) permanente, lesiones dérmicas y afectación hepática. OBJETIVO: Conocer la prevalencia de LN en paciente con BAVCC y la evolución clínica en un seguimiento a largo plazo. MÉTODOS: De enero de 1992 a diciembre 2017 se incluyeron paciente con BAVCC. La presencia de anticuerpos antinucleares anti-SSA/Ro y anti-SSB/La en suero materno confirmó LN. RESULTADOS: Ocho pacientes fueron incluidos con seguimiento de 10 ± 6 años, el 62.5 % con LN; dos fueron del sexo masculino. Uno diagnosticado in útero, dos al nacimiento, en ellos se implantó marcapaso; uno a los 12 años de edad y otro a los 15. Los otros dos casos fueron diagnosticados a los 18 y 26 años, se implantó marcapaso definitivo en ellos 8 y 5 años después respectivamente. En un caso no se implantó marcapaso definitivo; un recién nacido con solo un año de seguimiento. Al dar a luz, el 60 % de las madres estaban libres de enfermedad reumática y en conjunto todas tuvieron 19 hijos, ninguno de ellos presentó manifestaciones de LN. CONCLUSIONES: El BAVCC es raro y frecuentemente está asociado a una enfermedad autoinmune materna, prácticamente todos requerirán de marcapaso definitivo en alguna época de su vida.

Can prolonged P-R interval predict clinical outcomes in non-ST elevation acute coronary syndrome patients?

BACKGROUND: The present study aimed to respond to clinical question, can prolonged P-R interval predict clinical outcomes in non-ST elevation acute coronary syndrome patients? METHODS: This descriptive-analytical study was conducted on cardiac patients. All of the non-ST elevation acute coronary syndrome (NSTEACS) including non-ST elevation myocardial infarction (NSTEMI) and unstable angina patients included in the study. Then they divided into two groups: prolonged P-R interval and normal P-R interval. The patients who had a history of digoxin and calcium channel blocker use, using antiarrhythmic drugs, known valvular or congenital heart disease and connective tissue, unreadable P-R interval and cardiac block were excluded. Data were collected using the questionnaire consisted demographic data and clinical outcomes and a follow-up part was completed by one of the researchers. RESULTS: Finally, 248 patients completed the study. The results showed both of the two groups had significant differences in terms of the history of myocardial infarction (MI) (p = 0.018), the level of high-density lipoprotein (HDL) (p = 0.004), heart rate (p = 0.042), inverted T wave (p = 0.017), anterior ST- segment depression (p = 0.008), normal report of coronary angiography (CAG) (p = 0.003), three vessels disease (p = 0.043), left main lesion (p = 0.045) and SYNTAX score (p = 0.032) based on the CAG report. The results of six-month follow-up showed although, the frequency of ischemic stroke, coronary artery disease (CAD) and cardiovascular death were higher in prolonged P-R interval groups. The chi-square test showed this difference was statistically non-significant (p > 0.05). The multivariate logistic regression model revealed non-significant relationships between prolonged P-R interval and SYNTAX score, significant CAD, three-vessel disease, inverted T wave, anterior ST depression, heart rate and HDL. CONCLUSIONS: Based on the results of our study the six-month follow-up showed non-significant outcomes. Further studies are recommended to assess the long-term outcomes.

Boerhaave's Syndrome Presenting in the Setting of Third-Degree Heart Block.

Introduction: Boerhaave's syndrome, or the spontaneous transmural perforation of the esophagus, is typically thought to be due to an increase in esophageal pressure such as that which occurs during vomiting or retching. Another common etiology of esophageal perforation is esophageal instrumentation, such as during esophagogastroduodenoscopy or transesophageal echocardiography. This life-threatening condition requires prompt diagnosis and treatment to prevent patient demise. While a history of vomiting can aid in diagnosis, this history can be difficult to elicit in an unconscious patient or may be altogether absent. Additionally, Boerhaave's syndrome can present similarly to more common upper gastrointestinal or cardiac conditions. Since mortality increases with delays in diagnosis and treatment, it is imperative that clinicians maintain a high level of suspicion for Boerhaave's syndrome and initiate treatment urgently. Case Description: This report presents a 76-year-old man who presented to the emergency department after a history of several syncopal episodes and was found to be in complete heart block. Two days later, he acutely developed abdominal distention and coffee ground emesis. As the medical team was able to gather more history from the patient and his family, it was revealed that he had associated vomiting with his episodes of syncope. CT scan of the abdomen and pelvis demonstrated pneumomediastinum concerning for esophageal perforation. His clinical status subsequently deteriorated. He was intubated and a temporary transvenous pacer was placed before being transferred to our facility for emergent surgery. Discussion: Complete heart block in the setting of Boerhaave's syndrome is exceptionally rare, with only 2 cases reported in the literature. The decision to place a pacemaker in the setting of esophageal perforation/sepsis is complicated and depends on the patient's bacteremia status related to noncardiac comorbidities. Clearly this case represents the need for excellent multidisciplinary decision-making processes with excellent communication between hospital staff and all caretakers. Expeditious diagnosis and treatment of esophageal perforation is essential to prevent leaking of gastric contents into the mediastinum and worsening of cardiac complications and sepsis. Additionally, critical timing of various surgical procedures, especially the need for a permanent pacemaker implant with bacteremia is a complicated process not well described in the surgical literature.

Case report: Cardiac arrest after radiofrequency ablation in a 76-year-old male.

RATIONALE: Previous studies have found that the main treatment of sinus arrest is pacemaker treatment. It is rare to have 12 s of sinus arrest after radiofrequency ablation, and whether a permanent pacemaker is implanted immediately in this case is not described in the guidelines. PATIENT CONCERNS: A 76-year-old male patient with persistent atrial fibrillation (AF) developed sinus arrest lasting 12 s in the early morning of the fourth day after using radiofrequency ablation for pulmonary vein isolation. DIAGNOSIS: The patient was diagnosed with AF and sinus arrest. INTERVENTIONS: The patient received cardiopulmonary resuscitation, intravenous injection of atropine 1 mg, and intravenous infusion of isoproterenol 1mg and immediately recovered consciousness thereafter. Approximately, 1.5 h later, the patient underwent surgery to install a temporary pacemaker in the right femoral vein. OUTCOMES: The patient had repeated episodes of sinus arrest after the implantation of a temporary pacemaker. After 3 weeks, the patient stabilized and was discharged. The patient was followed up for 1 year and did not experience any recurrence of sinus arrest or AF. LESSONS: We consider the potential for postoperative myocardial edema, injury to the sinoatrial node during the procedure, propafenone poisoning, and autonomic dysfunction as contributors to the occurrence of sinus arrest after radiofrequency ablation. When sinus arrest occurs after radiofrequency ablation, we can choose the appropriate treatment according to the patient's condition.

Newborns with congenital complete heart block: Advice for perinatal care providers.

Congenital complete heart block (CCHB) is a rare, but a potentially life-threatening manifestation of autoimmune diseases in neonates. Bradycardia in CCHB can be misdiagnosed as foetal distress in utero and thus precipitating a Caesarean section. We report a case series of three neonates with bradycardia without any electrolyte abnormalities and structurally normal hearts with favourable outcomes.

Autoimmune congenital heart block: a case report and review of the literature related to pathogenesis and pregnancy management.

Autoimmune congenital heart block (ACHB) is a passively acquired immune-mediated disease characterized by the presence of maternal antibodies against components of the Ro/SSA and La/SSB ribonucleoprotein complex that mainly affects the cardiac conducting system. ACHB occurs in 2% of women with positive anti-Ro/SSA and anti-La/SSB antibodies and causes a high risk of intrauterine fetal death, neonatal mortality, and long-term sequelae. In this review, we first describe a case of ACHB to provide preliminary knowledge. Then, we discuss the possible pathogenic mechanisms of ACHB; summarize the pregnancy management of patients with positive anti-Ro/SSA and anti-La/SSB antibodies and/or rheumatic diseases, the prevention of ACHB, and the treatment of ACHB fetuses; and propose routine screening of these antibodies for the general population. Careful follow-up, which consists of monitoring the fetal heart rate, is feasible and reassuring for pregnant women with positive anti-Ro/SSA and/or anti-La/SSB antibodies to lower the risk of ACHB in fetuses. Moreover, maternal administration of hydroxychloroquine may be useful in preventing ACHB in pregnant women with anti-Ro/SSA and/or anti-La/SSB antibodies.

Severe cardiac conduction disease associated with titin gene mutation.

Heart block is rare in pediatrics with many possible causes. An association between complete heart block (CHB) and pathogenic titin (TTN) mutations have not been previously described. We report a 9-year-old female with history of leukodystrophy and family history of atrial fibrillation who presented with syncope and conduction abnormalities, including CHB. She underwent pacemaker implantation and genetic testing demonstrated a pathogenic TTN mutation likely responsible for her cardiac findings. Our case suggests an association between TTN mutations and conduction disease and emphasizes broadening gene testing in assessing these patients, especially when a family history is present.

Cardioneuroablation for pediatric patients with functional sinus node dysfunction and paroxysmal atrioventricular block.

INTRODUCTION: Severe transitory episodes of bradycardia with subsequent syncope in children are common, and generally portend a benign prognosis. Rarely, patients may experience prolonged asystolic episodes secondary to significant sinus pauses (SP) or paroxysmal atrioventricular block (AVB). Cardioneuroablation (CNA) is a catheter-based intervention, used to identify and ablate the epicardial ganglionated plexi (GP), which results in disruption of the vagal-mediated parasympathetic input to the sinus and atrioventricular node. OBJECTIVE: Describe the methodology and role of CNA for treatment of pediatric patients with functional AVB or SP. METHODS: This is a single-center, case series study. Patients with SP or AVB, 21 years of age or younger, who underwent CNA between 2015 and 2021 were included. CNA was performed via anatomically guided and high-frequency stimulation methods. RESULTS: Six patients were included. The median age was 18.9 years (range 12.3-20.9 years), 33% female. Two patients had prolonged SP, two had paroxysmal AVB, and two had both SP and AVB. Four patients had prior syncope. The median longest pause was 8.9 s (range 3.9-16.8) with 11 total documented pauses (range 2-231) during the 6 months pre-CNA. Post-CNA, the median longest pause was 1.3 s (range 0.8-2.2) with one documented SP after termination of atrial tachycardia at the 3-month follow-up. At 6 months, the median longest pause was 1.1 s (0.8-1.3) with 0 documented pauses. No patients had syncope post-CNA. CONCLUSION: CNA may be an effective alternative to pacemaker implantation in pediatric patients with syncope or significant symptoms secondary to functional SP or AVB.

Perivascular Basal Echogenicity in the Fetal Heart: An Unconventional Marker of Maternal Autoimmune Antibodies.

Fetal congenital heart block is a widely recognized complication of pregnancies with anti Ro/La antibodies. Less common presentations, including dilated cardiomyopathy, myocarditis, valvular insufficiency, and endocardial fibroelastosis, have also been described in these fetuses. We present two cases where recognition of isolated basal echogenicity of the fetal heart, an unconventional manifestation of anti-SSA antibodies during pregnancy, led to a suspicion and ultimately diagnosis of antibody positivity in the mother. These cases highlight the importance of being aware of this rare manifestation of anti-SSA antibodies during pregnancy, as early recognition of antibody positivity may lead to improve fetal and maternal outcomes.

Outcomes of atrioventricular septal defects with and without down syndrome: analysis of the national inpatient database.

BACKGROUND: Controversial data exist about the impact of Down syndrome on outcomes after surgical repair of atrioventricular septal defect. AIMS: (A) assess trends and outcomes of atrioventricular septal defect with and without Down syndrome and (B) determine risk factors associated with adverse outcomes after atrioventricular septal defect repair. METHODS: We queried The National Inpatient Sample using International Classification of Disease codes for patients with atrioventricular septal defect < 1 year of age from 2000 to 2018. Patients' characteristics, co-morbidities, mortality, and healthcare utilisation were evaluated by comparing those with versus without Down syndrome. RESULTS: In total, 2,318,706 patients with CHD were examined; of them, 61,101 (2.6%) had atrioventricular septal defect. The incidence of hospitalisation in infants with atrioventricular septal defect ranged from 4.5 to 7.5% of all infants hospitalised with CHD per year. A total of 33,453 (54.7%) patients were associated with Down syndrome. Double outlet right ventricle, coarctation of the aorta, and tetralogy of Fallot were the most commonly associated with CHD in 6.9, 5.7, and 4.3% of patients, respectively. Overall atrioventricular septal defect mortality was 6.3%. Multivariate analysis revealed that prematurity, low birth weight, pulmonary hypertension, and heart block were associated with mortality. Down syndrome was associated with a higher incidence of pulmonary hypertension (4.3 versus 2.8%, p < 0.001), less arrhythmia (6.6 versus 11.2%, p < 0.001), shorter duration for mechanical ventilation, shorter hospital stay, and less perioperative mortality (2.4 versus 11.1%, p < 0.001). CONCLUSION: Trends in atrioventricular septal defect hospitalisation had been stable over time. Perioperative mortality in atrioventricular septal defect was associated with prematurity, low birth weight, pulmonary hypertension, heart block, acute kidney injury, and septicaemia. Down syndrome was present in more than half of atrioventricular septal defect patients and was associated with a higher incidence of pulmonary hypertension but less arrhythmia, lower mortality, shorter hospital stay, and less resource utilisation.

Permanent pacemaker implantation after alcoholic septal ablation induced complete heart block: Long-term impact.

INTRODUCTION AND OBJECTIVES: Patients with hypertrophic obstructive cardiomyopathy (HOCM) that remain symptomatic despite optimized medical therapy often undergo alcohol septal ablation (ASA). One of the most frequent complications is complete heart block (CHB), requiring a permanent pacemaker (PPM) in variable rates of up to 20% of patients. The long-term impact of PPM implantation in these patients remains unclear. This study aimed to evaluate the long-term clinical outcomes in patients who implant PPM after ASA. METHODS: Patients who underwent ASA at a tertiary center were consecutively and prospectively enrolled. Patients with previous PPM or implantable cardio-defibrillator were excluded from this analysis. Patients with and without PPM implantation after ASA were compared based on their baseline characteristics, procedure data and three-year primary endpoint of composite of all-cause mortality and hospitalization and secondary endpoint of composite of all-cause mortality and cardiac cause hospitalization. RESULTS: Between 2009 and 2019, 109 patients underwent ASA, 97 of whom were included in this analysis (68% female, mean age 65.2 years old). 16 patients (16.5%) required PPM implantation for CHB. In these patients, no vascular access, pacemaker pocket or pulmonary parenchyma complications were noted. The baseline characteristics of comorbidities, symptoms, echocardiographic and electrocardiographic findings were identical in the two groups, with higher mean age (70.6±10.0 years vs. 64.1±11.9 years) and lower beta-blocker therapy rate (56% vs. 84%) in the PPM group. Procedure-related data showed higher creatine kinase (CK) peaks in the PPM group (1692 U/L vs. 1243 U/L), with no significant difference in the alcohol dose. At three years after ASA procedure, there were no differences in the primary and secondary endpoints between the two groups. CONCLUSIONS: Permanent pacemaker after ASA induced CHB do not affect long term prognosis in hypertrophic obstructive cardiomyopathy patients.

Clinical presentation, management, and survival in dogs with persistent atrial standstill in the United Kingdom.

OBJECTIVES: To investigate the clinical and echocardiographic presentation of dogs with persistent atrial standstill (PAS), identify variables measured at first presentation that could predict their survival, and document the progression of the disease after pacing. MATERIALS AND METHODS: Retrospective study of medical records of dogs diagnosed with PAS at three referral hospitals of the United Kingdom over seven years. RESULTS: Twenty-six dogs were diagnosed with PAS during the study period. Median age of the population was three years (range: seven months-12.5 years). The most common clinical sign was syncope (14/26). Twenty-four dogs received artificial pacemakers (PM). Major complications after PM implantation were observed in four dogs (four/24). Serial echocardiographic examinations showed that cardiac dimensions of PAS dogs with left atrial or left ventricular dilation at first presentation did not return to reference range after pacing. Further dilation of the cardiac chambers, recurrence of congestive heart failure (CHF), or development of new episodes of CHF were documented in seven, four, and 10 PAS dogs, respectively, despite pacing. Median survival time for cardiac-related deaths after PM implantation was 1512 days (18-3207). Neither CHF nor echocardiographic variables at presentation predicted survival after PM implantation in PAS dogs. CONCLUSIONS: Persistent atrial standstill (PAS) is an uncommon bradyarrhythmia, occurring in young adult dogs. Affected dogs were often presented with syncope. Whilst syncope resolved, cardiac remodeling persisted after PM implantation. Long-term survival was favorable after PM implantation and was not predicted by congestive status or cardiac chamber size at first presentation.

[Clinical characteristics of fetal cardiac disease in patients with anti-SSA antibody positive].

OBJECTIVE: To investigate the clinical manifestations and laboratory indicators of anti-Sjögren's-syndrome-related antigen A (SSA) antibody associated fetal cardiac disease. METHODS: Pregnant women hospitalized at Peking University People's Hospital from January 2013 to July 2023 were included. Eleven patients with anti-SSA antibody positive were eventually diagnosed with fetal cardiac di-sease. And patients with anti-SSA antibody positive without fetal cardiac disease were selected as controls. Clinical manifestations, laboratory indications and drug usage were compared between the two groups. RESULTS: Among these 11 patients, congenital heart block was confirmed in seven, which was the most common manifestations of fetal cardiac malformation. The proportion of the patients diagnosed with autoimmune disease before pregnancy in fetal cardiac malformation group was significantly lower than that in the control group (P=0.032), while most of the patients in the fetal cardiac malformation group received immune-related examinations for the first time because of this time's fetal cardiac diagnosis. While most of the patients in the control group received routine examinations because of autoimmune diseases diagnosed before pregnancy. During pregnancy, the white blood cell level [(9.29±2.58)×109/L vs. (7.10±1.90×109/L, t=3.052, P=0.004], erythrocyte sedimentation rate [(49.50 (48.00, 51.00) mm/h vs. 23.00 (15.00, 30.25) mm/h, Z=-2.251, P=0.024], IgA level [3.46 (2.30, 5.06) g/L vs. 2.13 (1.77, 2.77) g/L, Z=-2.181, P=0.029], and antinuclear antibody (ANA) titers [1∶320 (1∶160, 1∶320) vs. 1∶80 (1∶40, 1∶160), Z=-3.022, P=0.003] were significantly higher in fetal cardiac malformation group than in the control group. The proportion of positive anti-SSB antibody during pregnancy did not show a statistically significant difference between the two groups (37.5% vs. 7.7%, P=0.053). There was no significant difference in hydroxychloroquine dosage and initiation time between the two groups. The dosage of prednisone in the second and third trimesters was significantly higher in the cardiac malformation group than that in the control group, but there was no significant difference in the first trimester. CONCLUSION: Fetal cardiac disease is rare in pregnant women with anti-SSA antibody. White blood cell, erythrocyte sedimentation rate, IgA, the titer of ANA positivity were higher in the fetal heart disease group during pregnancy. Since congenital heart block is difficult to reverse, its prevention and monitoring are more important than remedial treatment.

Reverse complete heart block using transcutaneous pacing and repeated plasmapheresis in a neonate with lupus: a case report.

BACKGROUND: It has been reported that the complete heart block (CHB) in neonatal lupus (NL) cannot be reversed. This study reported a case of NL-CHB that was reversed by transcutaneous pacing and repeated plasmapheresis. CASE PRESENTATION: A 35+ 6-week male preterm baby was transferred to the neonatal intensive care unit of the Army Medical Center in May 2020 for slight cyanosis around the lips and nose. Two days after birth, a sudden decrease in heart rate was observed during electrocardiogram (EGG) monitoring. Physical examination revealed a bluish-purple discoloration around the lips and an irregular heartbeat. EGG showed the presence of isolated P (142 bpm) and QRS (78 bpm) waves, ventricular escape beats, and a diagnosis of NL-CHB. To reverse the condition, transcutaneous pacing and five sessions of plasmapheresis were performed. At a 1.5-year follow-up, the baby exhibited well-developed cardiac structure and normal neurodevelopment. CONCLUSIONS: Transcutaneous pacing and repeated plasmapheresis might be possible to reverse CHB in NL.

Cost of Pacing in Pediatric Patients With Postoperative Heart Block After Congenital Heart Surgery.

Importance: Surgical correction of congenital heart defects (CHDs) has improved the lifespan and quality of life of pediatric patients. The number of congenital heart surgeries (CHSs) in children has grown continuously since the 1960s. This growth has been accompanied by a rise in the incidence of postoperative heart block requiring permanent pacemaker (PPM) implantation. Objective: To assess the trends in permanent pacing after CHS and estimate the economic burden to patients and their families after PPM implantation. Design, Setting, and Participants: In this economic evaluation study, procedure- and diagnosis-specific codes within a single-institution database were used to identify patients with postoperative heart block after CHS between January 1, 1960, and December 31, 2018. Patients younger than 4 years with postoperative PPM implantation were selected, and up to 20-year follow-up data were used for cost analysis based on mean hospital event charges and length of stay (LOS) data. Data were analyzed from January 1, 2020, to November 30, 2022. Exposure: Implantation of PPM after CHS in pediatric patients. Main Outcomes and Measures: Annual trends in CHS and postoperative PPM implantations were assessed. Direct and indirect costs associated with managing conduction health for the 20 years after PPM implantation were estimated using Markov model simulation and patient follow-up data. Results: Of the 28 225 patients who underwent CHS, 968 (437 female [45.1%] and 531 male [54.9%]; 468 patients aged <4 years) received a PPM due to postoperative heart block. The rate of CHS and postoperative PPM implantations increased by 2.2% and 7.2% per year between 1960 and 2018, respectively. In pediatric patients younger than 4 years with PPM implantation, the mean (SD) 20-year estimated direct and indirect costs from Markov model simulations were $180 664 ($32 662) and $15 939 ($1916), respectively. Using follow-up data of selected patients with clinical courses involving 1 or more complication events, the mean (SD) direct and indirect costs were $472 774 ($212 095) and $36 429 ($16 706), respectively. Conclusions and Relevance: In this economic evaluation study, the cost of PPM implantation in pediatric patients was found to accumulate over the lifespan. This cost may represent not only a substantial financial burden but also a health care burden to patient families. Reducing the incidence of PPM implantation should be a focused goal of CHS.