Pregnancy in women with arrhythmogenic right ventricular cardiomyopathy/dysplasia

OBJECTIVES: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a cardiac disease characterised by myocardial necrosis followed by fibro-fatty substitution leading to the onset of ventricular arrhythmias. The aim of the present study was to analyse pregnancy in women affected by this condition. STUDY DESIGN: Six women affected by ARVC/D who underwent a pregnancy were studied with a follow-up programme, consisting of 12-lead ECG, signal-averaged ECG, 24-h ECG and two-dimensional and Doppler echocardiogram performed before the beginning of the pregnancy, at 3rd and 7th month of gestation and after the delivery. RESULTS: All women were on antiarrhythmic therapy during pregnancy; two complained of palpitations in the last 3 months. Delivery was performed at full terms in all, with caesarean section and epidural anaesthesia in four. Mean weight at birth was 3490g. No adverse reactions on the newborns were detected. All patients were advised against breast-feeding. No significant morphological changes were detected. During the period following the delivery (1-6years, mean 2,6years) one subject experienced a sustained ventricular tachycardia. CONCLUSIONS: Pregnancy seems to be well tolerated in patients affected by ARVC/D, but a programmed clinical protocol is mandatory particularly in the last trimester and puerperium, due to increased risk of ventricular arrhythmias.     

Arrhythmogenic right ventricular dysplasia in pregnancy: a case report

BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD) is characterized by progressive fibrous or fibrofatty tissue replacement of the right ventricular myocardium. Interspersed adipocytes and fibrous tissue may provide foci for arrhythmias. The clinical spectrum of ARVD may include asymptomatic premature ventricular complexes to ventricular tachycardia and sudden death. There is currently little information about ARVD in pregnancy. CASE: A 29-year-old primigravida, diagnosed with ARVD 1 year prior to pregnancy, underwent a full-term, uncomplicated pregnancy and delivery while maintained on acebutolol and an implanted cardioverter defibrillator. Her infant was born without an apparent cardiac anomaly or heart rate abnormality. CONCLUSION: Successful management of pregnancy complicated by ARVD can be accomplished with an implanted cardioverter defibrillator and an antiarrhythmic agent. Such patients should be managed with close monitoring during pregnancy for signs and symptoms of arrhythmia and preventive obstetric care appropriate to their clinical profile to optimize normal deliveries.     

Cardiac arrest and implantation of a cardioverter-defibrillator in a pregnant woman

During pregnancy increased incidence of maternal cardiac arrhythmias is observed. We reported a 31 year old primipara after two incidents of cardiac arrest due to ventricular fibrillation during pregnancy at 10th and 16th week of gestation. The implantable cardioverter defibrillator was implanted at the 19th week of gestation. There was no complication after ICD implantation. A healthy child was born by cesarean section at 38th week of gestation.     

Right ventricular hypertrophic cardiomyopathy in the fetus --may it be caused by chronic oral tocolysis with fenoterol?

The use of fenoterol in the treatment of preterm labor is associated with the risk of many complications in the mother and the fetus. We present a case of a multipara treated with oral fenoterol due to threatening preterm labor 14 weeks. At 35 weeks of gestation the fetus was diagnosed with hypertrophic cardiomyopathy with severe impairment of the right ventricle. The only factor that might have caused such a state of the fetal circulatory system was fenoterol, used from 21 weeks of gestation. After the withdrawal of the fenoterol the fetal right ventricular function improved gradually. However fetal cardiac hypertrophy persisted until the birth at 39 weeks of gestation. Concentric hypertrophy of the right ventricular wall and interventricular septum were confirmed in the newborn.     

Management of New-Onset Atrial Fibrillation in Pregnancy: When Should Early Delivery Be Considered?

BackgroundThe hemodynamic and physiological changes of pregnancy may predispose women to cardiac arrhythmias such as atrial fibrillation (AF). Nevertheless, new-onset AF in pregnancy remains rare, and treatment is challenging. Current recommendations are to treat pregnant women with AF as non-pregnant adults, by using pharmacological or synchronized electrical cardioversion, without mention of gestational age or possibility of delivery.CaseA 23-year-old nulliparous woman developed new-onset symptomatic AF at 36² weeks gestation, but presented to our hospital was delivered at 36⁴ weeks gestation. Beta-blockers were administered for heart rate control. After 48 hours, the decision was made to proceed with delivery rather than cardioversion. The patient's arrhythmia resolved spontaneously postpartum without further treatment.ConclusionIn pregnant patients near or at term, delivery should be considered in the management of new-onset AF after consultation with cardiology, anaesthesiology, and maternal-fetal medicine.     

Normal cardiac ventricular geometry and function during the last trimester of pregnancy and early neonatal period

Summary. Fetal and early neonatal left and right ventricular cardiac geometry and left ventricular cardiac function were studied by M-mode technique in a cross-sectional study between 28 and 40 weeks of gestation ( n =227) and a longitudinal study from 24 h before until 48 h after delivery ( n =15). Antenatally both ventricles were geometrically uniform; there was a 3·2–3·4-fold increase in left ventricular volume and a nearly 3-fold increase in left ventricular stroke volume and cardic output. During the early neonatal period the right ventricular transverse diameter reduced in size; there was a further 1·1-fold rise in left ventricular volume and a further 1·2–1·3-fold rise in left ventricular stroke volume and cardiac output.     

Prenatal constriction of the fetal ductus arteriosus--related to maternal pain medication?

Physiological fetal circulation requires patency of the ductus arteriosus. As gestation proceeds, the sensitivity of the ductus to dilating prostaglandins diminishes. The sensitivity to constricting agents like PGE-synthetase inhibitors, present in many analgetics, however, increases. Fetuses affected by an antenatal constriction of the ductus arteriosus (DC) may present with different signs of cardiac failure including dilated right ventricle, tricuspid regurgitation and abnormal venous Doppler. We report on four cases with prenatal DC, presenting at 34, 35, 36 and 37 weeks of gestation. They were referred to fetal echocardiography because of abnormal routine echo scans with unexplained signs of right heart decompensation. Three patients were medicated during pregnancy with either aspirin (low dose), metamizole or ibuprofen. One patient did not take any drugs, especially no pain medication drug in pregnancy. Immediate delivery was performed in all cases. The neonates were in a good condition; echocardiography showed different degrees of right heart hypertrophy which disappeared in all infants by the age of 3 months except in case 2. Unexplained fetal right heart decompensation requires detailed echocardiographic evaluation of the ductus arteriosus and a sophisticated medical history with regard to analgesics. In contrast to ibuprofen and high-dose aspirin, metamizole and low-dose aspirin have not yet been reported as possible agents constricting the fetal arterial duct. In any suspected context, early delivery as in our cases may save babies life. Any application of non-steroidal anti-inflammatory drugs in pregnancy requires close fetal follow-up due to their potentially life-threatening effect.     

Intrauterine hemodynamics of tricuspid valve dysplasia in a growth-restricted infant

We present a case of fetal tricuspid valve dysplasia, which was diagnosed at 22 weeks of gestation during a routine obstetrical examination. Serial fetal echocardiographic evaluation revealed progressive right ventricular outflow tract obstruction and persistent cardiomegaly. A female infant weighing 1,916 g was delivered by elective cesarean section at 38 weeks of gestation. Longstanding compression of the fetal lungs secondary to the persistent cardiomegaly resulted in severe respiratory distress and cyanosis immediately after birth. Although, mechanical ventilation and continuous infusion of prostaglandin were instituted, the infant died of respiratory failure at 21 days of age. During the pregnancy, the fetus exhibited intrauterine growth restriction, but hydrops did not occur. In regard to the fetal hemodynamics in this cardiac anomaly, transatrial communication is essential for fetal survival. The diameter of the fossa ovalis, which is a marker of transtrial blood flow, was adequate in this case. However, marked enlargement of the right heart associated with regurgitation interfered with left ventricular filling and output, which resulted in restriction of the combined ventricular output and intrauterine fetal growth restriction.     

Fetal Dilated Cardiomyopathy Associated With Variants of Uncertain Significance in MYH7 and DSG2 Genes: A Case Report and Review of the Literature

BackgroundFetal dilated cardiomyopathy (DCM) is an uncommon prenatal diagnosis associated with significant morbidity and mortality.CaseThis report describes a patient with a diagnosis of fetal DCM at 31⁰ weeks gestation, several weeks after a maternal flu-like illness. Spontaneous improvement was noted on serial echocardiograms. Maternal Coxsackievirus B titers were significantly elevated at 1:80, although post-natal cord blood test results were negative. Genetic panel testing for DCM demonstrated two heterozygous variants of uncertain significance in the MYH7 and DSG2 genes. Although an early post-natal echocardiogram demonstrated a normal left ventricular ejection fraction, right ventricular dysfunction was noted with subsequent cardiac decompensation requiring temporary inotropic support. An echocardiogram at the age of 2 years confirmed normal biventricular function.ConclusionThe finding of fetal DCM should trigger a broad evaluation. In the setting of limited fetal cardiac reserve, the significant hemodynamic changes that occur post-natally may trigger additional decompensation. Clinicians should be aware of the prognostic value of right ventricular function, as measured by fractional area change, in addition to the limitations of serologic and genetic testing.     

Evaluation of the human fetal cardiac size and function

Two-dimensional-directed M-mode echocardiography was done on 80 normal fetuses between the 17th to 42nd weeks of gestation. The M-mode beam transected the ventricles at the level of the chordae tendineae at the tip of atrioventricular valves. Right and left ventricular dimensions and free wall thicknesses correlated well with gestational age. Calculated measurements showed a good correlation of the stroke volume and cardiac output with gestational age. The right ventricular dimension, however, was significantly greater than the left ventricular one. Fractional shortening of the right and left ventricle did not change significantly with advancing gestational age. This study indicates that the human fetal right ventricle dimension, stroke volume, and cardiac output are slightly larger than that of the left ventricle. This study also suggests that the human fetus increases its cardiac output to match its growth and it does so by increasing ventricular size rather than fractional shortening or heart rate.     

In utero progressive pulmonary stenosis successfully treated with transcatheter intervention after delivery.

It is unclear whether pulmonary stenosis with intact ventricular septum is a secondary cardiac malformation. We report an infant with pulmonary stenosis (diagnosed by fetal echocardiography) with progressive obstruction in late gestation who presented with increasing transvalvular pressure gradients (15 mm Hg at 22 weeks' gestation to 47 mm Hg at 35 weeks). The tricuspid/mitral valve annulus ratio decreased from 1.25 at 24 weeks' gestation to 0.96 at 33 weeks. At 38 weeks' gestation, a male infant weighing 3,524 g, with Apgar scores of 9 and 9 at 1 and 5 minutes, respectively, was delivered by cesarean section. Critical pulmonary stenosis was confirmed by postnatal catheterization. These findings support the postulation that pulmonary stenosis is a progressive disorder. After percutaneous balloon dilatation, the transvalvular pressure gradient decreased and the right ventricular cavity increased gradually. The transvalvular pressure gradient had decreased to 15 mm Hg and the tricuspid/mitral valve annulus ratio was 0.93 at the age of 2 years.     

Early prenatal management of a fetal ventricular tachycardia treated in utero by amiodarone with long term follow-up

Fetal cardiac arrhythmias are one of the causes of intra-uterine congestive heart failure and non-immune hydrops fetalis leading to fetal death. As ventricular tachycardia (VT) is rarely diagnosed in utero, it leads to emergency deliveries. We report a prenatal diagnosis of fetal tachycardia at 20 weeks of gestation associated with non-immune hydrops fetalis. The tachycardia seemed to be supraventricular and was initially treated by digoxin and sotalol. The hydrops increased and sotalol was stopped in order to give the mother a high dose of amiodarone by mouth over a long period. Although the tachycardia, which the ECG recorded at birth revealed to be of ventricular origin, persisted but at a lower rate, the new treatment proved successful. The child is three years old now and health, though with persistent VT. In conclusion, fetal tachycardia with similar ventricular and atrial rates can be a VT and the drug of choice in this case seems to be amiodarone.     

Biometry of the fetal heart between 10 and 17 weeks of gestation

OBJECTIVES: Assessment of the dimensions of the cardiac chambers and the great arteries in the human fetus may be helpful in the prenatal diagnosis of congenital heart disease. The purpose of this prospective cross-sectional study was to compile normative data in fetal cardiac measurements in early pregnancy. The structure of the fetal heart was examined in 136 normal singleton fetuses between 10 and 17 weeks of gestation. METHODS: The transversal heart diameter, both ventricular dimensions, interventricular septal thickness, heart area, heart circumference, thoracic diameter, thoracic circumference and thoracic area were measured in the four-chamber view during diastole. Diameters of the pulmonary trunk and ascending aorta were obtained in the short axis and long axis view during systole. Ultrasound examinations were performed with a 5.0-MHz transvaginal and/or transabdominal phased-array sector scanner. RESULTS: The four-chamber view and the cross-over of the pulmonary artery and the aorta were adequately visualized in 44% of the fetuses at 10 weeks of gestation, in 75% at 11 weeks of gestation, in 93% at 12 weeks of gestation and in 100% of the fetuses at 13-17 weeks of gestation. Before 14 weeks of gestation transvaginal sonography was superior to the transabdominal sonography in visualization of the fetal heart and great arteries. After 14 weeks of gestation transabdominal sonography accurately demonstrated the structure of the fetal heart. The ratio of right and left ventricle (RV/LV) and the ratio of the pulmonary trunk and aorta (PT/AO) were constant during this period of gestation (approximately 1.00 and 1. 10, respectively). The ratio of the cardiac and thoracic area showed only a slight increase with advancing gestational age, but with significant correlation. The fetal heart rate showed a slow decrease from 167 to 150 bpm in this period of gestation. The transversal heart diameter, both ventricular dimensions, interventricular septal thickness, heart area, cardiothoracic diameter ratio, aortic diameter and the pulmonary trunk diameter showed a highly significant linear correlation to the gestational age and the biparietal diameter. CONCLUSION: The advancing quality of ultrasound images allows fetal echocardiography in the first and early second trimester. Our normative data could be the basis of studying the development of cardiac structures in congenital heart disease and it might be helpful in the detection of some congenital heart defects in early pregnancy.     

Incidence and management of arrhythmias in women

There are gender-specific differences in the nature and frequency of specific cardiac arrhythmias. Sex-specific variations in the electrophysiologic structure of the heart and/or hormonal effects on modulating ionic channel function may help to explain some of these differences, such as the increased prevalence of symptoms of congenital long QT syndrome in women, and the increase in episodes of supraventricular tachycardia in the perimenstrual period in susceptible patients. On the other hand, women suffer only 20% of sudden cardiac deaths. Fewer women than men have been enrolled in clinical trials for the treatment of ventricular arrhythmias because of difficulty in recruiting female patients, exclusion of women due to comorbid conditions, and/or the later appearance of coronary artery disease in females compared to males. Management of arrhythmias in women is often different from that in men, as women are less likely to receive a dual-chamber pacemaker and to have automatic defibrillator implantation, even when clinically appropriate. These differences may be due to the smaller size of women, a difference in the frequency of comorbid conditions, and patient preference, with women more likely to decline aggressive therapy. This article discusses the differences in presentation and the frequency of specific arrhythmias in women, and suggests measures to improve arrhythmia management in the female patient.     

Prenatal catheter placement for fetal cystic adenomatoid pulmonary malformation: a case report

INTRODUCTION: Fetal congenital cystic adenomatoid malformation is a pulmonary developmental anomaly arising from an overgrowth of the terminal respiratory bronchioles. This is such a rare malformation that it is not always thought of as a diagnostic possibility. CASE: In the present case, after a large pulmonary cyst started deviating the mediastinum and following an increase in amniotic fluid volume in the fetus at 28 weeks of gestation, the placement of a catheter for continuous drainage was performed. Subsequent follow-up with ultrasounds and serial echocardiograms revealed normalization of the position of mediastinum, normal amniotic fluid levels, and the correct position of the catheter. The size of the cyst was decreasing daily, but 8 weeks after fetal invasive procedure, there was concern about the stabilization of its size, with the suspicion of obstruction of the catheter. Cesarean delivery was performed at 38 weeks' gestation. The size of the cyst on the day of C-section was 3.0 x 2.9 x 2.1 cm. At delivery, the infant weighed 3,030 g, with no evidence of respiratory distress, revealing the correct location of catheter. At day 4 after delivery a superior right pulmonary lobe resection was performed. CONCLUSION: Large pulmonary cysts may produce deviation of mediastinum and can lead to cardiac tamponade, nonimmune hydrops and pulmonary hypoplasia. Precise prenatal imaging and different fetal therapeutic strategies may allow survival of affected fetuses.     

Prenatal diagnosis of isolated congenitally corrected transposition of the great arteries

Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac defect characterized by the atria connecting with anatomically discordant ventricles and the ventricles connecting with discordant and transposed great arteries, which allows hemodynamic compensation. Most patients with ccTGA have associated intracardiac anomalies, which could be a diagnostic clue, whereas isolated forms are infrequently diagnosed during the neonatal period and in utero. We describe a fetus that was diagnosed with ccTGA and without additional cardiac anomalies at 25 weeks of gestation. The parallel course of the great arteries discovered during a routine obstetric scan indicated this rare cardiac anomaly. Further detailed examination of the ventricular morphology helped to confirm the diagnosis. Despite hemodynamic compensation, the long-term prognosis of ccTGA is uncertain because of the possible development of arrhythmias or heart failure later in life. Our findings showed that fetal echocardiography can detect prenatal ccTGA.     

Magnetocardiography-guided management of an unusual case of isoimmune complete atrioventricular block complicated by ventricular tachycardia

A fetus who was diagnosed at 25 weeks of gestation with isoimmune AV block presented at 34 weeks with a precipitous fall in ventricular rate and periods of tachycardia. Magnetocardiography revealed the tachycardia to be ventricular. After delivery, nonsustained ventricular tachycardia continued. The baby then successfully paced, and at higher ventricular rates the tachycardia resolved. Five years later the child has normal ventricular function and is doing well.     

In utero treatment of fetal complete heart block with terbutaline. A case report.

BACKGROUND: Isolated fetal complete heart block is an uncommon finding, with a mortality rate of 20-30%. Various treatment modalities have been reported, with no consistent success. CASE: Fetal complete heart bock was diagnosed in a 30-year-old woman at 29 weeks' gestation. She had an elevated antinuclear antibody and anti-SSA antibody titer. Fetal cardiac decompensation was detected at 32 weeks. After a trial of intravenous isoproterenol without significant side effects, she was treated with oral terbutaline. An increase in the fetal ventricular rate and complete resolution of hydrops fetalis occurred. A male infant was delivered by cesarean section, at term, and underwent cardiac pacemaker implantation at 4 days of age. CONCLUSION: The treatment of fetal complete heart block is controversial. Premature delivery with cardiac pacing is associated with high morbidity and mortality. We report prenatal treatment with oral sympathomimetic medication, which allowed delivery to be delayed until term, with a successful outcome.     

Prenatal diagnosis of fetal complete atrioventricular block with QT prolongation and alternating ventricular pacemakers using multi-channel magnetocardiography and current-arrow maps

We report a case of fetal complete atrioventricular block (CAVB) with QT prolongation and alternating ventricular pacemakers diagnosed by magentocardiography (MCG). Fetal bradyarrhythmia of around 60 bpm was detected at 30 weeks of gestation. Ultrasonography revealed fetal CAVB without structural cardiac anomalies. MCG was recorded at 30 weeks of gestation using a 64-channel superconducting quantum interference device (MC-6400, Hitachi, Japan). The averaged MCG revealed QT prolongation, and the current-arrow maps generated by MCG revealed alternating ventricular pacemakers. The diagnosis was confirmed by electrocardiogram after birth. A permanent pacemaker was implanted during the early neonatal period, and the infant was healthy at 6 months of follow-up. MCG may be a useful diagnostic tool for fetal CAVB with QT prolongation and alternating ventricular pacemakers.     

Fetal screening of congenital heart disease

Echocardiograms of two hundred and ninety normal fetuses at 18-39 weeks of gestation were studied with a real-time linear scanner (3.5 MHz). Aortic valvular diameter, pulmonary valvular diameter, mitral valvular diameter, tricuspid valvular diameter and total cardiac dimension were measured. These parameters increased with fetal growth. The ratios of pulmonary to aortic valvular diameter and of tricuspid to mitral valvular diameter were more than 1, suggesting right ventricular dominance in the fetal hearts. The cardio-thoracic ratio was about 50% in this period. The four-chamber view, left ventricular long-axis view, ventricular short-axis view, short-axis view of the great arteries, the view of the aortic arch and the view of the inferior and superior vena cava were also recorded. These six views could be obtained in 9.8% of all subjects at the same time. Detection rates for these views were the greatest in pregnancy at 22-33 (especially 28-29) weeks of gestation. The ventricular short-axis view and short-axis view of the great arteries were easily detected in the dorsoposterior position, whereas the view of the aortic arch was obtained in the dorsoanterior position. It was concluded that fetal echocardiography needed to be performed at least twice, at 22-23 and 28-29 weeks of gestation, and this would be useful in diagnosing some congenital heart diseases.