Complete congenital heart block in the offspring of an asymptomatic woman with isolated high titer anti-ro antibody

@#Among pregnant women, 1-2% are anti-Ro positive and while half of them have symptoms of connective tissue disease, the rest are asymptomatic. The presence of anti-Ro is of concern because of the risk of congenital heart block in the child. We report the case of an asymptomatic 27-year-old G2P1(1001) woman, who presented with persistent fetal bradycardia in her 21st week of gestation (AOG) and was found to have elevated titers for anti-Ro (>320 U/ml). Hydroxychloroquine 200 mg/day and prednisone 10 mg/day were given from the 33rd week of gestation up until the delivery. At 37 weeks AOG, she delivered a live male neonate with a complete heart block. On the 6th day of life, the infant remained bradycardic, hence a pacemaker was inserted and heart rate maintained at 100-120 bpm. On subsequent follow-ups, the mother and child did not develop any systemic manifestations and the infant was thriving well. While a diseased condition may not be apparent in a pregnant anti-Ro positive woman, the risk of neonatal lupus (NL) is demonstrated in this patient’s case. This report illustrates how prenatal care of an asymptomatic woman led to the discovery of a fetal abnormality and served to prepare the family and the medical team to ably handle the birth and subsequent care of a neonate with NL.

Short term outcomes with dual chamber versus single chamber pacing for atrioventricular block - A crossover trial

A total of 42 patients were studied for primary outcomes of quality of life and 6MWD between VVIR and DDD modes. At end of 2 months after device implantation, randomization was done and the device was programmed to VVIR or DDD modes. At the end of 2 months in this mode QOL and functional was assessed and the patient was switched to other mode. The same protocol was followed at the end of 2 months. We found no difference in functional capacity and quality of life between the two pacing modes. None of the patients developed pacemaker syndrome and there was no preference for any of the modes.

Complete Heart Block Due to Coronary Air Embolism during Trans-catheter Closure of Atrial Septal Defect

Coronary air embolism is a dreadful complication of cardiac intervention despite careful prevention. In literature reported complications of coronary air embolism ranges from clinically non significant events to cardiogenic shock, myocardial infarction and death. We report a case of massive coronary air embolism in right coronary artery, which results in hypotension and complete heart block in a 33 -year-old female undergoing elective closure of atrial septal defect (ASD) by percutaneous transcatheter intervention. The patient stabilized after timely measures like oxygen support, dopamine infusion, cardiac compression and repeated bolus injection of heparinized saline. She then underwent successful percutaneous ASD device closure.

Ruptured Sinus of Valsalva Aneurysm Presenting as Complete Heart Block - A Rare Presentation

The dissection of the sinus of Valsalva aneurysm into the interventricular septum leading to complete heart block is a rare complication with only few cases reported in the literature. We report a case of young farm laborer, who presented with multiple episodes of syncope. Upon clinical evaluation, we found third-degree atrioventricular block due the rupture of right sinus of Valsalva into the interventricular septum.

Transvenous Low Septal Permanent Pacing for a Patient with Ebstein’s Anomaly – A Case Report

Patients with Ebstein’s anomaly can present with bradyarrhythmias at any age. In view of the abnormal tricuspid valve anatomy associated with this condition, pacing these individuals can present with various challenges. We present a patient with Ebstein’s anomaly

Hammock Bridge on Fire: Complete AV Block in a Patient with Congenitally-corrected Transposition of the Great Arteries and Wolff-Parkinson-White Pre-excitation Pattern.

Aims: To recognize heart block as a complication associated with congenitally-corrected transposition of the great arteries (CCTGA). Presentation of the Case: A healthy 36 year old male with CCTGA presented with syncope as a manifestation of heart block. A unique feature of this case was the presence of an accessory pathway that served as the atrio-ventricular conducting structure for more than 3 decades. Discussion: rationale for a cardiac resynchronization device (CRT) as opposed to a simple pacemaker system is emphasized in this case. Conclusion: clinicians must be aware of heart block a frequent complication of CCGTA. CRT appears as a more favorable option than a single systemic ventricular pacemaker in such patients.

Transvenous Permanent Pacemaker Implantation in 14 Month Old baby with Congenital Complete Heart Block.

Congenital Complete Heart Block in utero has been diagnosed more frequently with the clinical use of fetal echocardiography. The fetus with complete heart block may remain asymptomatic or develop congestive heart failure. Pacemaker therapy in children involves unique issues regarding patient size, growth, development and possible presence of congenital heart disease. Historically, epicardial pacing was more common in children. As technology has improved, generators and leads have become smaller and more advanced, allowing transvenous pacing system in children. Pacemaker therapy is even technically feasible in infants and neonates. We present a 14mth old baby of just 7.5kg who presented to us with Adams Stokes and subtle signs of congestive heart failure that after stabilization was implanted a transvenous MRI compatible permanent pacemaker (VVIR).

Cardiac pacing in left bundle branch/ bifascicular block patients.

The primary concern in patients with bifascicular block is the increased risk of progression to complete heart block. Further, an additional first-degree A-V block in patients with bifascicular block or LBBB might increase the risk of block progression. Anesthesia, monitoring and surgical techniques can induce conduction defects and bradyarrhythmias in patients with pre-existing bundle branch block. In the setting of an acute MI, several different types of conduction disturbance may become manifest and complete heart block occurs usually in patients with acute myocardial infarction more commonly if there is pre-existing or new bundle branch block. The question that arises is whether it is necessary to insert a temporary pacing catheter in patients with bifascicular block undergoing anesthesia. It is important that an anesthesiologist should be aware of the indications for temporary cardiac pacing as well as the current recommendations for permanent pacing in patients with chronic bifascicular and trifascicular block. This article also highlights the recent guidelines for temporary transvenous pacing in the setting of acute MI and the different pacing modalities that are available for an anesthesiologist.

Complete Heart Block due to Lithium Toxicity / 대한정신약물학회지

Lithium treatment has been associated with a wide range of cardiac complications. We observed a 53-year-old female patient who presented with complete heart block due to lithium toxicity. The patient had been diagnosed as schizoaffective disorder and had been taking a stable dose of lithium, 1,500 mg/day since January 2007. Recently, she begun a strict diet and experienced muscle weakness and lethargy a few days later. While receiving fluid therapy, she lost her consciousness and was transferred to an emergency medical center. An electrocardiogram revealed that she had complete heart block, so a temporary pacemaker was inserted immediately. After 4 days of intensive care, her heartbeat recovered spontaneously and the temporary pacemaker was removed. On the 11th day, she had sufficiently recovered and could ambulate by herself. Lithium levels were measured at 5.22 mEq/L and 0.66 mEq/L on the 1st and 4th day of treatment, respectively. This case illustrates the importance of educating patients and their relatives about the possible lithium toxicity caused by a strict diet.

Complete Heart Block due to Lithium Toxicity / 대한정신약물학회지

Lithium treatment has been associated with a wide range of cardiac complications. We observed a 53-year-old female patient who presented with complete heart block due to lithium toxicity. The patient had been diagnosed as schizoaffective disorder and had been taking a stable dose of lithium, 1,500 mg/day since January 2007. Recently, she begun a strict diet and experienced muscle weakness and lethargy a few days later. While receiving fluid therapy, she lost her consciousness and was transferred to an emergency medical center. An electrocardiogram revealed that she had complete heart block, so a temporary pacemaker was inserted immediately. After 4 days of intensive care, her heartbeat recovered spontaneously and the temporary pacemaker was removed. On the 11th day, she had sufficiently recovered and could ambulate by herself. Lithium levels were measured at 5.22 mEq/L and 0.66 mEq/L on the 1st and 4th day of treatment, respectively. This case illustrates the importance of educating patients and their relatives about the possible lithium toxicity caused by a strict diet.

Complete Heart Block In Mumps Myocarditis.

Myocarditis is a relatively common complication occurring in Mumps (epidemic parotitis). Inflammation of the myocardium causes complications in some of these cases. Observation of complete heart block in patients of acute mumps myocarditis is a rare instance. We report a case of complete heart block in a patient suffering from mumps.

Outcome of pregnant mothers with systemic lupus erythematosus (focusing on congenital heart block) / 소아과

PURPOSE: Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. METHODS: The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. RESULTS: There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). CONCLUSION: The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.

The Clinical Characteristics and Outcome of Neonates born to Systemic Lupus Erythematosus Mothers

PURPOSE: Neonatal lupus erythematosus (NLE) is a rare disorder caused by the transplacentally transmitted maternal autoantibodies. It is characterized by congenital complete atrioventricular block (CCAVB), skin lesion and less commonly hepatic and hematologic abnormalities. This study was designed to investigate the clinical characteristics and outcome of systemic lupus erythematosus (SLE) mothers and their neonates. METHODS: 29 neonates born to 25 SLE mothers were admitted to the Neonatal Intensive Care Unit of Chonnam University Hospital between January 1994 and June 2005. We investigate the medical records retrospectively. RESULTS: Among 25 SLE mothers, 32.4% had history of spontaneous abortion or stillbirth in previous pregnancy, and 7 (28.0%) of preeclampsia and 3 (12.0%) of premature rupture of membranes in present pregnancy. Among 29 newborns, 14 (48.2%) were premature, 4 (13.7%) were intrauterine growth retardation, and 11 (37.9%) were confirmed as NLE. Anti-SSA/Ro was positive in 13 (52.0%) of 25 examined neonates including 6 (85.7%) of 7 neonates with electrocardiographic abnormalities. Two cases of CCAVB were diagnosed during the pregnancy. One patient didn't show heart failure, he is on the followed up without medical treatment until 4 years of age. The other one showed heart failure, cardiac pacemaker was inserted at 7 days of age, dilated cardiomyopathy developed at 4 years of age. Five neonates with sinus bradycardia resolved spontaneously. One (3.4%) had typical lupus skin lesion with thrombocytopenia and elevated liver enzyme, one (3.4%) had leukopenia, and two (6.9%) had elevated liver enzymes only. CONCLUSIONS: With careful antenatal monitoring of SLE mothers and their fetuses, neonates should be monitored by autoantibody, EKG, and laboratory test.

Clinical characteristics and treatment results on 50 complete heart block cases

Study on the significant symptomatic and effect of treatment methods on 50 patients with 3 degree heart block (23 females and 27 males), were treated at Cardiovascular Hospital during 2002-2003. The results: 70% of total patients had Adams-stokes cyncope. 28% of them were made extent or temporary pacemaker with indications as slow heart rate making a severe Adams-stokes syncope or nearly making Adams-stokes syncope, A-V disorders, young patients with suspective inflammatory induced A-V block. All of patients were treated by Atropine, Theophylin, Ephedrin or Isoprenalin, but only 2 cases decreased of A-V block. 38 patients had successful with permanent pacemaker implantation

Clinical characters and result treatment of 50 cases with idiopathic complete A-V block

To study the significant symptoms and effect of treatment methods on complete heart block. Methods and results: 50 patients (23 female and 27 male) with complete A-V block were studied. 70% of total patients had Adams-stokes syncope. 28% of them were dazzled, dizzied, chest-pain... only one case was without symptom. Average heart rate was 39 beat per minute. 7 patients (14%) had heart failure. All of patients were treated by Atropine, Theophyline, Ephedrine or Isoprenalin, but only 2 cases decreased of A-V block. 38 patients had successful with permanent pacemaker implantation (PPI)

Bloqueo auriculoventricular completo fetal / Fetal complete atrioventricular block

Uno de los disturbios más graves del ritmo cardíaco fetal es el bloqueo aurículoventricular completo o de 3er grado (BAVC), condición de fácil detección clínica y ecocardiográfica, y muchas veces de altísima mortalidad fetal, que indica la necesidad de intervención terapéutica urgente. Se presenta el caso clínico de una paciente que teniendo el antecedente de un RN anterior con el mismo diagnóstico, se envía a nuestro servicio con bradiarritmia en el feto actual, para realizar estudio y tratamiento. Se comenta además frecuencia, etiopatogenia, diagnóstico y tratamiento.

One of the worst fetal cardiac arrhythmias is a complete atrioventricular block or third degree block. This disorder is easy to detect clinically or by ultrasound, having a high fetal mortality rate making a quick therapeutic intervention necessary. This is a clinical case of a pregnant woman with a previous infant born with this condition, that was send to as, presenting a fetal bradyarrhythmia, for assessment and treatment. Also, pregnancy, pathogenesis, diagnosis and therapy are analized.

A case of neonatal lupus syndrome with congenital complete heart block / 대한산부인과학회잡지

The neonatal lupus syndrome is characterized by skin lesions, hepatic and hematologic abnormalities and congenital heart block. Congenital heart block which is believed to be caused by transplacental passage of the anti-Ro (SSA)/La (SSB) antibodies from mother to infant, is known to occur in 1 in 20,000 live births. In contrast to other manifestation of neonatal lupus syndrome, which usually subside within 6 months after birth, congenital heart block is a permanent and potentially fatal complication. We experienced a case of neonatal lupus syndorme with congenital complete heart block in a newborn of asyptomatic mother with anti-Ro (SSA)/ La (SSB) antibodies.

A Case of Congenital Complete Heart Block

In complete heart block(CHB), there is a complete failure of the atrial impulse leading into a ventricular response, the atria and ventricles beat independently, with the latter having a slower rate. Approximately one-third of infants with congenital CHB have associated structural heart disease such as corrected transposition of the great arteries, single ventricle, and the heterotaxy syndrome. For those patients without associated anomalies in whom bradycardia causes heart failure unresponsive to drugs, a pacemaker is necessary. We report a case of congenital complete heart block showing a ventricular beat of 57 /min despite atrial beat of 125/min. The mother of the baby was asymptomatic but her serologic tests were positive for connective tissue disease. The patient had positive serologic tests for anti-Ro antibody and anti-La antibody but associated structural heart anomalies were not found. He remained well without signs and symptoms of heart failure and no treatment was required except 02 supply.

Two Cases of Neonatal Arrhythmia Observed by Fetal Echocardiography / 대한주산의학회잡지

M-mode, pulsed Doppler and Doppler color flow mapping, in addition to two-dimensional echocardiography, have greatly improved imaging of the fetal heart through identification of abnormal cardiac anatomy and rhythm in utero. The early detection of cardiac disturbance in utero permits alteration in obstetric management such as delivery in a high-risk center for optimal neonatal care and/or decision in optimal delivery time. We report two cases of the neonatal arrhy-thmia which were observed by fetal echocardiography. In the first case, female baby showed neonatal arrhythmias including tachycardia and brady-cardia until 3 days after birth, and then turned to bradyarrhythmia due to non-conducted atrial bigeminy. These events lead us to review the fetal echocardiographs of the patient carefully. Premature atrial contractions were observed in her fetal echocardiography. At 2 months after birth, the patient's electrocardiogram showed normal sinus rhythm. Severe neonatal bradycardia of the second case was due to congenital complete heart block, identified clearly by electrocardiogram after birth. This case also showed complete heart block in her fetal echocardiography. After insertion of the temporary pacemaker, cardiomegaly was improved. Both the neonate and the mother had positive anti-SSA/Ro autoantibody. But any other symptoms and signs of neonatal lupus did not appear in the neonate. Patient's mother also did not show any symptoms and signs of systemic lupus erythematosus. Since the prognosis depends upon the cause of bradyarrhythmia in fetus and neonates, differential diagnosis is important in obstetric management and optimal neonatal treatment.

Characteristics of Subsidiary Pacemaker in Complete Heartblock with Narrow QRS Complex

BACKGROUND: The stability of A-V junctional rhythm is the most important clinical interest in the patients with the complete heart block with narrow QRS complex. The QRS morphology, the basal heart rate & the reponse of subsidiary pacemaker to drugs may be helpful to assess the stability. But it can not be assessed exactly. The recording of the His-bundle was thought to be a means of predicting the clinical course of the patients and the necessity of the artificial pacemaker insertion;also it can assess the A-V block site. But it is invasive study cannot assessed exactly the stability of subsidiary pacemaker. To investigate the characteristics of subsidiary pacemaker, we studied the responses of the artificial pacemaker to ventricular overdrive suppression and the drugs by used the artificial pacemaker in the patient with the complete heart block with the narrow QRS complex, which can be accepted as "stabli" in general. METHOD: There was total of 11 patients diagnosed with the complete AV block with the narrow QRS complex(the escape junctional rhythm was less than 0.10sec) at the Yeungnam University Hospital from May 1988 to June 1989. To investigate the ventricular overdrive suppression of subsidiary pacemaker, the response of the subsidiary pacemaker after IV administatin of drugs (atropine, isoproterenol, lidocaine) and the junctional recovery time were measured. RESULTS: Thtee patterns were obtained in the overdrive suppression test (fig.1). The recovery time of the subsidiary pacemaker was 364.5+/-212.15% of the basal R-R interval. Among seven of the eight patients who showed syncoped, the JRTmax were 3 times higher than the basal R-R interval. In one of eight, CJRT which represent the absolute time, was prolonged to 560 msec. In one case, we examined overdrive suppression test serially was which shows gradual decrement of junctional recovery time, and normal sinus rhythm was recovered at the 14th after the development of complete heart block. After VOS, the junctional recovery time of the subsidiary pacemaker was 364.5+/-212.1% of basal R-R interval in complete heart block group. After injection of atropine, the decrement of R-R interval was 5.51+/-3.88% of basal R-R interval. After injection of isoproterenol, the decrement of R-R interval was 32.76+/-8.09% of basal R-R interval. Marked increase of the basal heart rate was observed. After injection of lidocaine, the increment of R-R interval was 1.72+/-1.82% of basal R-R inteval. CONCLUSION: The results suggest that measurement of the junctional recovery time following overdrive suppression is useful for evaluation of stability of subsidiary pacemaker. And also the subsidiary pacemaker shows the better response to sympathomimetic agent than to parasympathetic blocker.