Complete congenital atrioventricular block. Prenatal diagnosis and perinatal management

OBJECTIVE: To describe our experience in prenatal diagnosis and perinatal management of congenital atrioventricular heart block, as well as pacemaker treatment in the neonate. MATERIAL AND METHODS: A total of 13 fetuses are included. The diagnosis of atrioventricular dissociation was established by Doppler heart rate sample in the right atrium to show the atrial activity while the sample in the Aorta reflected the ventricular heart rate. Gestational age at diagnosis, ventricular heart rates, autoimmune maternal pathology, maternal blood tests for autoantibodies antiRo+, congenital structural heart disease, fetal hydrops, maternal medical treatment, perinatal results and pacemaker neonatal implantation are described. RESULTS: Gestational age at diagnosis ranged between 22 and 32 (mean 27.6) weeks. Ventricular heart rates ranged between 32 to 80 (mean 54) beats/min. AntiRo+ antibodies were detected in 5 mothers, and clinical systemic lupus erythematosus was found in only one. Four had congenital heart disease (2 ventricular inversion and corrected TGA, 1 complete atrio-ventricular canal and 1 tricuspid atresia). Signs of heart failure and hydrops were detected in 9 fetuses. Treatment with beta-metasona and ritodrine was administered to 7 mothers when the ventricular heart rate dropped below 60 beats/min. Intrauterine fetal death occurred in 3 fetuses with structural congenital heart disease and hydrops. Delivery was performed by cesarean section in 8 preterm fetuses (one them a twins), 3 spontaneous deliveries at term and 3 stillbirth. Postnatal pacemaker implantation was carried out in 9 newborns (3 cases with unicameral temporal right ventricle electrode and 6 cases with permanent bicameral electrodes implanted through the subclavian vein and DDD pacemaker). Follow-up of the bicameral pacemaker group was satisfactory. CONCLUSION: Persistent fetal bradycardia is the first sign to diagnose prenatal complete atrioventricular heart block. Echocardiography asses fetal haemodynamic status and may detect signs of fetal deterioration. Hydrops and further drop in the ventricular heart rate warrant urgent cesarean section and pacemaker management of the newborn.     

Congenital heart block and pacemaker implantation (author's transl)

A six years old boy was sent to our service due to a loss of conscience spell. He had no preceding history E.K.G. showed a complete A-V block. Laboratory findings were within normal limits. Cardiac catheterism was performed and it ruled out any antomic abnormality, making evident a good contraction ability. To decide pacemaker implantation we evaluted: 1. That cardiac frequency was not increased by exercise neither by action of pharmacologyc agents (atropine, insoproterenol).-2. The existence of wide and bizarre QRS ocmplex.-3. And dizziness spels. The patient has been controlled during the past two years. Parasistolia has appeared, not showing modification till today.     

Mitochondrial encephalomyopathy (Kearns-Sayre syndrome) with complete atrioventricular block: a case report

A pacemaker was implanted into a 17-year-old man with cardiac failure due to complete atrioventricular block complicated by mitochondrial encephalomyopathy (Kearns-Sayre syndrome). Due to the possible complication of latent myocardial dysfunction, it was decided to implant the dual chamber pacemaker (DDD) and the operation mode was set to DDD 70 ppm 1 year after implantation; this alleviated the cardiac failure. In this case, the necessity of preventive pacemaker implantation in the early stage of cardiac failure was recognized.     

Right-heart endocarditis after pacemaker implantation as a cause of a round pulmonary infiltrate

HISTORY AND CLINICAL FINDINGS: A 57-year-old man developed intermittent fever and fatigue 11 months after a two-chamber cardiac pacemaker had been implanted because of 2 degrees and 3 degrees A-V block. Antituberculosis treatment was initiated as tuberculosis was suspected. The infiltrate regressed, but the other symptoms persisted. Four months later he was admitted as an emergency because of septicaemia. INVESTIGATIONS: In addition to a recently discovered cardiac murmur there was a raised erythrocyte sedimentation rate (116 mm) and leucocytosis (13 600/microliters) with shift to the left. Coagulase-negative staphylococci were grown on several blood cultures. Transoesophageal echocardiography (TOE) demonstrated vegetation on the tricuspid valve, the pacing wires and the right ventricular outflow tract. DIAGNOSIS, TREATMENT AND COURSE: After the diagnosis of infective endocarditis had been established, antibiotic treatment was give with imipenem, gentamycin and teicoplanin, the pacemaker system and adherent thrombotic material were removed and a DDD pacemaker implanted from the other side. The patient remained free of symptoms during a follow-up period of 12 months. INTERPRETATION: Delayed diagnosis of infective endocarditis is not uncommon, because of the scarcity of typical symptoms. Repeated blood cultures and TOE are essential for the diagnosis.     

Target-promoted alkylation of DNA

For 13 years between 1980 and 1992, 23 patients needed pacemaker (PM) implantation because of bradyarrhythmia, 21 of atrial fibrillation and two of complete A-V block, after valve surgery. Five of 88 survivors (5.7%) after OMC, 11 of 227 (4.8%) after MVR, one of 169 (0.6%) after AVR, five of 67 (7.4%) after MVR + AVR and one of 15 (6.7%) after TVR underwent PM implantation in postoperative period. Two cases who had heart failure was implanted PM in early postoperative period. In late period, the mean duration between previous valve surgery and PM implantation was 6.4 years in 12 cases after initial valve surgery and 2.3 years in nine after second valve surgery. Postoperative course after PM implantation was almost good, but one case was died due to critical arrhythmia. And one case underwent re-MVR because of mitral bioprosthesis dysfunction and one, without anticoagulant after OMC, was complicated cerebral infarction.     

Influenza vaccines

Varicella pneumonia can endanger the life of pregnant women. We have a case of varicella pneumonia complicating pregnancy in the third trimester. The patient required intubation, early treatment with acyclovir (700 mg/IV every eight hours), as well as the extraction of the fetus by cesarean section before the time gestation was completed. Early treatment with acyclovir has improved hope for these patients.     

An autopsy case of the sinus of Valsalva aneurysm involved with tuberculous inflammation, leading to complete heart block

A case is presented of unruptured aneurysm of the non coronary sinus of Valsalva, causing involvement of A-V node and right coronary artery compression. The patient was a 68 year-old woman with a complaint of general fatigue. Electrocardiogram showed complete A-V block. Computed tomography showed an aneurysm of the non coronary sinus of Valsalva. A temporary pacemaker was implanted, but the patient developed respiratory failure and heart failure and died. At autopsy, macroscopically disseminated tuberculosis was noted in both lungs and kidneys. Microscopically a tuberculous inflammatory lesion extending into the A-V node was found. We report this rare case with some consideration of the literature.     

Two cases of pulmonary dirofilariasis in Nagasaki Prefecture

BUT: Assess risk factors and pathogenesis of myocardial infarction in young women and evaluate cardiologic and obstetrical management of myocardial infarction during pregnancy as well as assess the prognosis of pregnancy in patients with a prior myocardial infarction. METHODS: Fra a personal observation of pregnancy after myocardial infarction, we reviewed the literature on two distinct themes: myocardial infarction during pregnancy and pregnancy after myocardial infarction. RESULTS: Pregnancy was deliberately authorized in a 21-year-old who had myocardial infarction 3 years earlier. The pregnancy was uneventful and cesarean section with epirual analgesia at 37 weeks gestation delivered a healthy 2,350 g boy. Unfortunately the patient died at her home 18 month later. Acardiac cause was retained although death due to ventricular rhythm disorder or recurrent myocardial infarction could not be confirmed due to lack of an autopsy. The prognosis of pregnancies after myocardial infarction would not appear to be catastrophic although this case raises some doubts as to whether pregnancy should be authorized in these patients. The major difference in mortality according to whether the infarction occurs before or during pregnancy would suggest that risk might be reduced by a haling period after the infarction. CONCLUSION: It appears that pregnancy in women with a prior myocardial infarction would be possible if sufficient precautions are taken. All situations increasing cardiac work should be avoided. Stress and pain must be avoided during delivery and the post-partum period. Intensive cardiological and obstetrical supervision supervision are required for correct management of the pregnancy.     

The McBride operation of hallux valgus in adolescents (author''s transl)

A 23 year old previously healthy man was stabbed in the anterior chest. This resulted in a ventricular septal defect and complete atrioventricular (A-V) block. The electrocardiogram revealed complete A-V block with a QRS pattern of right bundle branch block. His bundle recordings 26 days later revealed A-V dissociation with split His potentials (P-H1 interval of 100 msec and H2-V interval of 40 msec). During the study the escape QRS shifted from right to left bundle branch block with H2 potentials still preceding each QRS interval with H2-V intervals of 40 msec. A permanent pacemaker was implanted because of persistent congestive heart failure and bradycardia due to A-V block. The patient subsequently became asymptomatic. He died suddenly 3 1/2 years later. Pathologically there were sizable openings in both the tricuspid and mitral valve substance and a ventricular septal defect involving the pars membranacea and part of the adjacent muscular septum. Serial sections of the conduction system revealed total destruction and fibrous replacement of the bifurcation and beginning of the right and left bundle branches and subtotal fibrous replacement of the branching bundle. Thus, the bifurcation of the bundle of His was totally absent at autopsy despite apparent electrophysiologic evidence of its existence 26 days after the stab wound. A possible explanation for this discrepancy is the subsequent fibrosis of the bifurcation produced by hemodynamic changes at the lower margin of the ventricular septal defect.     

Bilateral renal artery stenosis diagnosed during pregnancy

Bilateral renal artery stenosis was diagnosed noninvasively in the 17th gestational week, in a chronically hypertensive pregnant woman, by renal artery duplex ultrasound examination, MRI and MR angiography. Continuous monitoring of the mother and the fetus was performed. Blood pressure was stabilized by complex antihypertensive therapy, but from the beginning of the third trimester superimposed preeclampsia developed gradually. In the 34th gestational week a 1600-g newborn was delivered by elective cesarean section. The case report draws attention to the significance of the thorough examination of hypertensive women before pregnancy.     

Results of atrioventricular synchronous pacing with optimized delay in patients with severe congestive heart failure

To verify that atrioventricular (AV) synchronous pacing (DDD) with short AV delay improves the condition of patients with severe congestive heart failure, we implanted DDD pacemakers in 10 patients with severe heart failure (New York Heart Association [NYHA] class III to IV). One day after pacemaker implantation, the AV delay was optimized by Doppler echocardiographic measurements over the aortic outflow tract. Patients were evaluated regarding NYHA class, stroke volume, cardiac output, ejection fraction, and quality of life at 1, 3, and 6 months after pacemaker implantation. Although the optimized AV delay was associated with short-term improvement in stroke volume and cardiac output (baseline stroke volume = 22 +/- 7 ml, day 1 = 28 +/- 12 ml; p = 0.03: baseline cardiac output = 1.9 +/- 0.6 L/min, day 1 = 2.2 +/- 1.1 L/min; p = 0.10), the mean stroke volume, cardiac output, NYHA class, and ejection fraction did not change significantly after 1, 3, and 6 months of pacing compared with baseline values. Three patients improved in NYHA class during the follow-up. A consistent improvement in stroke volume, cardiac output, NYHA class, and ejection fraction was observed in only 1 patient. In conclusion, we found no beneficial effects of AV-synchronous pacing with optimized AV delay in patients with severe heart failure.     

Ultrasonographic appearance of non-Hodgkin''s lymphoma in a pregnant woman

Non-Hodgkin's lymphoma during pregnancy is rare. We report a case of non-Hodgkin's lymphoma detected in a pregnant women. She was initially diagnosed as having an ovarian tumor by ultrasonography, but definitive diagnosis was made at the time of cesarean section. After delivery she took combined chemotherapy for 7 cycles and is in complete remission over 1 year.     

Indications, effectiveness, and long-term dependency in permanent pacing after cardiac surgery

The purposes of this study were to define predictors of long-term pacemaker dependency in patients having permanent pacemakers implanted shortly after cardiac surgery, and to evaluate short- and long-term results and survival in this population. Data from 120 adult patients with implantation since 1980 were retrospectively analyzed. Acute and chronic complication rates (4.2% and 16.6%, respectively) were not higher than those expected in the general paced population. In addition, continuous rhythm was evaluated by use of pacemaker inhibition in a subgroup of 20 patients to verify the validity of clinical criteria for pacemaker dependency. Of the patients evaluated for dependency, 41% eventually became nondependent. Prolonged monitoring with an inhibited pacemaker confirmed the accuracy of our method of clinical evaluation of pacemaker dependency. Significant predictors of long-term pacemaker dependency were complete atrioventricular block as the indication and bypass time of > 120 minutes (by multivariate and univariate analyses, respectively). Postoperative complete atrioventricular block is the most important predictor of pacemaker dependency, enabling an earlier decision on permanent pacemaker implantation (no later than the sixth and the ninth postoperative days for wide-complex and narrow-complex escape, respectively). Further prospective studies are needed to define optimal implantation times for indications other than complete atrioventricular block.     

Sinus node dysfunction after complete correction of tetralogy of Fallot. Report of a case

Atrioventricular conduction defects after complete correction of tetralogy of Fallot are well known and their prevalence is decreasing with improved operative techniques. On the other hand, sinus node dysfunction has not been described after this type of surgery. The authors report the case of a 24 year old patient operated at the age of 2 for a favourable form of tetralogy of Fallot. In the long-term, this patient had a minimal anatomical sequel (mild pulmonary insufficiency) and first degree AVB with complete right bundle branch block with sinus bradycardia on the surface ECG. A recent aggravation of the patient's functional status led to rhythmological investigation which showed chronotropic atrial incompetence with nodal atrioventricular delay. The patient was implanted with a permanent DDD cardiac pacemaker programmed in the DDDR mode with a good outcome. Atrial chronotropic incompetence is one of the modes of expression of sinus node dysfunction. This has not been previously described in this context. This form of sinus node dysfunction should be systematically excluded after surgical cure of tetralogy of Fallot by exercise stress testing and 24 hour Holter ECG. Symptomatic patients are greatly improved by permanent cardiac pacing with hysteresis.     

Leptin and interleukin-6 in sepsis

Adenomyosis is a common pathologic finding significantly related to the menstrual and reproductive characteristics of women. Although noted during younger reproductive years, it usually presents in women over 35 years of age. For those with a strong desire to preserve fertility, there is presently no uniform agreement on the most appropriate therapeutic methods to manage the condition. Herein, we present a case of long-term secondary infertility with successful pregnancy after treatment of deep adenomyosis with cytoreductive surgery and a subsequent six-month course of gonadotropin-releasing hormone agonist (GnRHa) therapy. For those who want to conceive, early combined GnRHa therapy immediately following cytoreductive surgery and a delay of four to six months before attempting to fall pregnant is advisable. This is because adenomyosis tends to recur rapidly and the myometrium can be significantly disrupted during surgery. The major obstetric complications, such as uterine atony, rupture or placenta accreta, do not increase with adenomyosis during pregnancy. Although two events of threatened abortion and one of preterm labor were encountered during the pregnancy course, a healthy 2,900-g female was delivered by low transverse cesarean section at term. A cesarean section was performed because of previous large cytoreductive surgery. In contrast to GnRHa therapy alone, we report an effective alternative to hysterectomy in order to maintain fertility and achieve successful pregnancy.     

Moyamoya disease and pregnancy: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Many female patients with moyamoya disease are of childbearing years, including those who were diagnosed before entering their childbearing years. However, there have been no extensive reviews of the management of pregnancy and delivery in association with moyamoya disease. The purpose of this report is to describe the case of a patient with moyamoya disease complicated by pregnancy and to review the literature on other such cases. CLINICAL PRESENTATION AND INTERVENTION: We report a 23-year-old primipara with moyamoya disease who delivered uneventfully by cesarean section under spinal anesthesia at 38 weeks of gestation. In the literature, 30 cases were reported of patients who had been diagnosed with moyamoya disease before pregnancy and delivery, and 23 patients who were symptomatic and were diagnosed for the first time with moyamoya disease in association with pregnancy. CONCLUSION: There is no evidence that pregnancy increases the risk of cerebrovascular accident or that bypass surgery decreases its risk. Poor prognosis of the patient or the newborn is mostly caused by cerebral hemorrhage and not by cerebral ischemia. It is important to control blood pressure and especially to avoid toxemia during pregnancy. Either cesarean section or vaginal delivery can be accomplished safely. Any anesthetic method can be used, provided special attention is given to avoiding hypocapnia, hypotension, and hypertension. Oral contraceptives should be avoided.     

The concurrent implantation of a last-generation automatic defibrillator and a 2-chamber pacemaker

BACKGROUND: Potentially lethal interactions between concomitantly implanted pacemaker and automatic first-generation cardioverter-defibrillator have been reported. We therefore evaluated the reliability and safety of simultaneous implantation of dual-chamber pacemakers with a fourth generation cardioverter-defibrillator incorporating an improved arrhythmia detection system. METHODS: Concomitant implantation of an automatic cardioverter defibrillator and a dual-chamber pacemaker was performed in four patients for malignant tachyarrhythmias and symptomatic bradycardia. Two Jewel 7219C and two Micro Jewel 7223 (Medtronic Inc.) implantable cardioverter defibrillators were connected to an endocardial lead (Medtronic Mod. 6936). In 2 patients with a previously implanted cardioverter-defibrillator, the pacemaker was connected to bipolar atrial and ventricular leads. In the 2 patients who already had a dual-chamber pacemaker, the unipolar pacing leads implanted previously were used. At the time of implantation of the second device, interaction testing was performed evaluating ventricular fibrillation detection during high-output asynchronous pacing. Testing for malfunctions, reprogramming or variations of pacing thresholds of the pacemakers after electric shocks was also carried out. Moreover, intracavitary signals, stimulation thresholds and electrical therapies delivered were evaluated during follow-up. RESULTS: During interaction testing, induced ventricular fibrillation was detected, with no delay, in 4/4 cases (100%). After a mean follow-up of 6.3 +/- 5 months (range 2-13), 5 shocks for ventricular fibrillation, 10 shocks for fast ventricular tachycardia and 72 antitachycardia pacings were delivered. All these electrical therapies were considered appropriate. No symptomatic sustained tachyarrhythmia remained undetected. CONCLUSIONS: These data suggest that concomitant implantation of a fourth-generation automatic cardioverter-defibrillator and a dual-chamber pacemaker using either unipolar or bipolar leads can be performed safely. Extreme caution and rigorous interactions testing is nevertheless advised when considering this device combination.     

Aspects of transport of pet animals

BACKGROUND: Neonatal risk in herpes infections is very high: in the USA it is 1:2,000, in Japan 1:15,000 and in England 1:65,000. These differences seem to be due mostly to the different risk factors associated to the mother infection. The best strategy to cure herpes infection is to examine every patient during pregnancy and to identify the high risk ones. METHODS: In this study, 1,860 pregnant women have been submitted to a colposcopic and cytologic screening for the research of HSV. RESULTS: HSV was found in twenty (1.07%) women. Two groups have been made: the first with 14 HSV positive women at 3-6 months of pregnancy and second with 6 HSV positive women at eight months of pregnancy. Both groups were treated with acyclovir per os. The 14 women of the first group underwent vaginal delivery. The women of the second group underwent cesarean section. Only in 1 case a neonatal infection has been observed.     

Percutaneous transhepatic cholelithotripsy for difficult common bile duct stones

Laparoscopic myolysis, a procedure designed to shrink uterine myomas by coagulating their blood supply, is an alternative to myomectomy or hysterectomy in women who do not contemplate childbearing. Three patients conceived within 3 months after myolysis against the surgeon's advice. In two of these women the uterus ruptured at 32 and 39 weeks' gestation, respectively, associated with death of the 32-week fetus. The third patient had an uneventful elective cesarean section at 39 weeks' gestation. Until the risk of uterine rupture after myolysis has been accurately compared with that after myomectomy, women should not undergo myolysis if they wish to conceive. Should pregnancy occur after myolysis, caution and intensive surveillance of mother and fetus must be applied, and cesarean section should be performed at earliest signs and symptoms of uterine rupture and at term before onset of labor.     

Role of the preaxillary flora in pacemaker infections: a prospective study

BACKGROUND: Infection remains a severe complication after pacemaker implantation. The purpose of our prospective study was to evaluate the role of the local bacteriologic flora in its occurrence. METHODS AND RESULTS: Specimens were collected at the site of implantation for culture from the skin and the pocket before and after insertion in a consecutive series of patients who underwent elective permanent pacemaker implantation. Microorganisms isolated both at the time of insertion and of any potentially infective complication were compared by using conventional speciation and ribotyping. There were 103 patients (67 men and 36 women) whose age ranged from 16 to 93 years (mean+/-SD, 67+/-15). At the time of pacemaker implantation, a total of 267 isolates were identified. The majority (85%) were staphylococci. During a mean follow-up of 16.5 months (range, 1 to 24), infection occurred in four patients (3.9%). In two of them, an isolate of Staphylococcus schleiferi was recognized by molecular method as identical to the one previously found in the pacemaker pocket. In one patient, Staphylococcus aureus, an organism that was absent at the time of pacemaker insertion, was isolated. In another patient, a Staphylococcus epidermidis was identified both at the time of pacemaker insertion and when erosion occurred; however, their antibiotic resistance profiles were different. CONCLUSIONS: This study strongly supports the hypothesis that pacemaker-related infections are mainly due to local contamination during implantation. S schleiferi appears to play an underestimated role in infectious colonization of implanted biomaterials and should be regarded as an important opportunistic pathogen.