The multidisciplinary management of a mechanical mitral valve thrombosis in pregnancy: a case report and review of the literature.

Background: The management of anticoagulation for mechanical heart valves during pregnancy poses a unique challenge. Mechanical valve thrombosis is a devastating complication for which surgery is often the treatment of choice. However, cardiac surgery for prosthetic valve dysfunction in pregnant patients confers a high risk of maternofetal morbidity and mortality. Case summary: A 39-year-old woman in her first pregnancy at 30 weeks gestation presented to hospital with a mechanical mitral valve thrombosis despite therapeutic anticoagulation with low-molecular-weight heparin. She underwent an emergent caesarean section followed immediately by a bioprosthetic mitral valve replacement. This occurred after careful planning and organization on the part of a large multidisciplinary team. Discussion: A proactive, rather than reactive, approach to the surgical management of a mechanical valve thrombosis in pregnancy will maximize the chances of successful maternal and fetal outcomes.

Canadian Cardiovascular Society: Clinical Practice Update on Cardiovascular Management of the Pregnant Patient.

The number of women of childbearing age with cardiovascular disease (CVD) is growing because of increased survival of children with congenital heart disease. More women are also becoming pregnant at an older age, which is associated with increased rates of comorbidities including hypertension, diabetes, and acquired CVD. Over the past decade the field of cardio-obstetrics has significantly advanced with the development of multidisciplinary cardio-obstetric programs (COPs) to address the increasing burden of CVD in pregnancy. With the introduction of formal COPs, pregnancy outcomes in women with heart disease have improved. COPs provide preconception counselling, antenatal and postpartum cardiac surveillance, and labor and delivery planning. Prepregnancy counselling in a COP should be offered to women with suspected CVD who are of childbearing age. In women who present while pregnant, counselling should be performed in a COP as early as possible in pregnancy. The purpose of counselling is to reduce the risk of pregnancy to the mother and fetus whenever possible. This is done through accurate maternal and fetal risk stratification, optimizing cardiac lesions, reviewing safety of medications in pregnancy, and making a detailed plan for the pregnancy, labor, and delivery. This Clinical Practice Update highlights the COP approach to prepregnancy counselling, risk stratification, and management of commonly encountered cardiac conditions through pregnancy. We highlight "red flags" that should trigger a more timely assessment in a COP. We also describe the approach to some of the cardiac emergencies that the care provider might encounter in a pregnant woman.

Atrial arrhythmias in adults with congenital heart disease.

BACKGROUND: Atrial arrhythmias increase disease burden in the general adult population. Adults with congenital heart lesions constitute a rapidly growing group of patients with cardiovascular disease. We hypothesized that atrial arrhythmias increase with age and impair health outcomes in this population. METHODS AND RESULTS: We conducted a population-based analysis of prevalence, lifetime risk, mortality, and morbidity associated with atrial arrhythmias in adults with congenital heart disease from l983 to 2005. In 38 428 adults with congenital heart disease in 2005, 5812 had atrial arrhythmias. Overall, the 20-year risk of developing atrial arrhythmia was 7% in a 20-year-old subject and 38% in a 50-year-old subject. More than 50% of patients with severe congenital heart disease reaching age 18 years developed atrial arrhythmias by age 65 years. In patients with congenital heart disease, the hazard ratio of any adverse event in those with atrial arrhythmias compared with those without was 2.50 (95% confidence interval, 2.38 to 2.62; P<0.0001), with a near 50% increase in mortality (hazard ratio, 1.47; 95% confidence interval, 1.37 to 1.58; P<0.001), more than double the risk of morbidity (stroke or heart failure) (hazard ratio, 2.21; 95% confidence interval, 2.07 to 2.36; P<0.001), and 3 times the risk of cardiac interventions (hazard ratio, 3.00; 95% confidence interval, 2.81 to 3.20; P<0.001). CONCLUSIONS: Atrial arrhythmias occurred in 15% of adults with congenital heart disease. The lifetime incidence increased steadily with age and was associated with a doubling of the risk of adverse events. An increase in resource allocation should be anticipated to deal with this increasing burden.

Irreversible Acquired Noncompaction Cardiomyopathy in a Parturient with Corrected Atrial Septal Defect: A Case Report and Clinical Implications.

Left ventricular noncompaction (LVNC) is described as a cardiomyopathy with an increase in left ventricle trabeculations and recesses. We report a rare case of persistent pregnancy-acquired LVNC cardiomyopathy and review the anesthetic peripartum management strategies. A 33-year-old parturient was followed closely by the high-risk obstetric service for her second pregnancy. She had an unresolved LVNC cardiomyopathy that was diagnosed during her first pregnancy for which she had a caesarean section. Her symptoms included occasional palpitations and dyspnea. She was started on metoprolol and enoxaparin. A successful caesarean section was performed at 37 weeks gestation under regional anesthesia. Echocardiograms prior to and during the second pregnancy demonstrated persistence of the LV hypertrabeculations, LV systolic dysfunction, and a left ventricular ejection fraction (LVEF) of 35%. Pregnancy-induced LV hypertrabeculations occur in a significant proportion of women, but most cases spontaneously resolve completely. Favorable maternal and fetal outcomes require multidisciplinary care and careful selection of the anesthetic technique and drugs that maintain stable hemodynamics.

Secular trends in pregnancy rates, delivery outcomes, and related health care utilization among women with congenital heart disease.

BACKGROUND: The number of women with congenital heart disease (CHD) of reproductive age is increasing, yet a description of trends in pregnancy and delivery outcomes in this population is lacking. OBJECTIVE: To assess secular trends in pregnancy rates, delivery outcomes, and related health care utilization in the adult female CHD population in Quebec, Canada. METHODS: The Quebec CHD database was used to construct a cohort with all women with CHD aged 18-45 years between 1992 and 2004. Pregnancy and delivery rates were determined yearly and compared to the general population. Secular trends in pregnancy and delivery rates were assessed with linear regression. The cesarean delivery rate in the CHD population was compared to the general population. Predictors of cesarean section were determined with multivariable logistic regression. Cox regression, adjusted for comorbidities, was used to analyze the impact of cesarean sections on 1-year health care use following delivery. RESULTS: About 14 878 women were included. A total of 10 809 pregnancies were identified in 5641 women, of whom 4551 (80%) and 2528 (45%) experienced at least one delivery and/or abortion, respectively. Absolute yearly numbers and rates of pregnancies and deliveries increased during the study period (P < .05). The increment in cesarean section rates was more pronounced among women with CHD than among the general population. Gestational diabetes (OR 1.50, 95% CI [1.13, 1.99]), gestational hypertension (OR 1.81, 95% CI [1.27, 2.57]), and preeclampsia (OR 1.59, 95% CI [1.11, 2.8]) were independent predictors of cesarean delivery. Cesarean sections were associated with postpartum cardiac-hospitalization within 1 year following delivery (HR = 2.35, 95% CI [1.05, 5.28]). CONCLUSIONS: Yearly numbers and rates of pregnancies and deliveries in adult females with CHD rose significantly during the study period. Cesarean sections led to increased health care utilization. Further research is required to determine causes of high cesarean section rates in this patient population.

A nationwide evaluation of spontaneous coronary artery dissection in pregnancy and the puerperium.

OBJECTIVE: Spontaneous coronary artery dissection (SCAD) is a rare and potentially lethal cause of myocardial infarction (MI). The purpose of our study was to estimate the prevalence and maternal outcomes of pregnancies complicated by SCAD. MATERIALS AND METHODS: A population-based cohort study on all births identified in the Healthcare Cost and Utilization Project from 2008 to 2012. Disease prevalence was calculated and logistic regression was used to estimate the adjusted odds ratio (aOR) for risk factors and different maternal complications. RESULTS: A total of 4 363 343 pregnancy-related discharges were evaluated. 79 cases of SCAD were identified resulting in a prevalence of 1.81 per 100 000 pregnancies. The mean maternal age at the time of diagnosis was 33.4 years (±5.2). Chronic hypertension (aOR, 2.67; 95% CI 1.18 to 6.03), lipid profile abnormalities (aOR, 48.22; 95% CI 24.25 to 95.90), chronic depression (aOR, 3.56; 95% CI 1.43 to 8.83) and history of migraine (aOR, 3.93; 95% CI 1.52 to 10.17) were associated with an elevated risk for SCAD. MI was diagnosed in 66 (85.5%) cases of SCAD with anterior and subendocardial territories being the most common locations. Thirty one patients (40%) with SCAD underwent angioplasty with the majority receiving stents, which was associated with a longer hospital stay than those treated conservatively or with bypass. CONCLUSIONS: SCAD is a rare aetiology of MI; risk factors and outcomes are illustrated in the current study. The puerperium is an important period for the development of pregnancy-related SCAD. Careful evaluation of pregnant and postpartum women with chest pain is warranted, especially if these risk factors are identified.

Mirror image atrial dilatation in adult patients with atrial fibrillation and congenital heart disease.

BACKGROUND: Atrial fibrillation (AF) is largely regarded to be initiated from left atrial (LA) dilatation, with subsequent dilatation of the right atrium (RA) in those who progress to chronic AF. We hypothesized that in adult patients with right-sided congenital heart disease (CHD) and AF, RA dilatation will predominate with subsequent dilatation of the left atrium, as a mirror image. METHODS: Adult patients with diagnosis of right-sided, ASD or left-sided CHD who had undergone an echocardiographic study and electrocardiographic recording in 2007 were included. RA and LA area were measured from the apical view. AF was diagnosed from a 12-lead electrocardiogram or Holter recording. A multivariate logistic regression model was used to identify predictors of AF and linear regression models were performed to measure relationship between RA and LA area and AF. RESULTS: A total of 291 patients were included in the study. Multivariate analysis showed that age (p=0.0001), RA (p=0.025) and LA area (p=0.0016) were significantly related to AF. In patients with pure left-sided pathologies, there was progressive and predominant LA dilatation that paralleled the development of AF from none to paroxysmal to chronic AF. In patients with pure right-sided pathologies, there was a mirror image of progressive and predominant RA dilatation with the development of AF. CONCLUSION: We observed a mirror image atrial dilatation in patients with right sided disease and AF. This may provide novel mechanistic insight as to the origin of AF in these patients and deserves further studying in the form of targeted electrophysiological studies.

Stenosis of the superior limb of the systemic venous baffle following a Mustard procedure: an under-recognized problem.

BACKGROUND: Patients with atrioventricular concordance and ventriculoarterial discordance (DTGA) and a Mustard procedure may develop stenosis of the superior limb of the systemic venous baffle (SLSVB). The frequency of this complication in an adult cohort was evaluated. METHODS: Patients >18 years with DTGA and a Mustard procedure with and without a pacemaker (PM)/implantable cardioverter defibrillator (ICD) were identified through an institutional database. Subjects were included following a cardiac imaging study (computed tomography, magnetic resonance imaging, venography or cardiac catheterization) and follow-up in the PM/ICD or congenital cardiac clinics from 2001 to 2007. The primary end-point was narrowing of the SLSVB (<10mm) on cardiac imaging. Hemodynamically significant narrowing was defined by: azygous vein dilatation with retrograde flow or superior vena cava syndrome or the need for dilatation ± stenting of the SLSVB. RESULTS: Narrowing of the SLSVB was observed in 49/112 patients (70 males) age 31 ± 6 years (range 18-49) and was hemodynamically significant in 15/49. Of 29 patients with a PM (23) or ICD (6) and cardiac imaging, 17 had narrowing of the SLSVB which was hemodynamically significant in 8. Non-echocardiographic imaging had a sensitivity of 88% at detecting narrowing of the SLSVB in contrast to pulse-wave Doppler, which yielded a sensitivity of 16% (61% negative predictive value, 88% positive predictive value). CONCLUSIONS: In our adult cohort of Mustard patients, narrowing of the SLSVB had a prevalence of 44% and was more likely to be detected by non-echocardiographic imaging. Baffle patency should be evaluated before transvenous device implantation.

Atrial arrhythmias in adult patients with right- versus left-sided congenital heart disease anomalies.

Atrial arrhythmias (AAs) are a common complication in adult patients with congenital heart disease. We sought to compare the lifetime prevalence of AAs in patients with right- versus left-sided congenital cardiac lesions and their effect on the prognosis. A congenital heart disease diagnosis was assigned using the International Disease Classification, Ninth Revision, diagnostic codes in the administrative databases of Quebec, from 1983 to 2005. Patients with AAs were those diagnosed with an International Disease Classification, Ninth Revision, code for atrial fibrillation or intra-atrial reentry tachycardia. To ensure that the diagnosis of AA was new, a washout period of 5 years after entry into the database was used, a period during which the patient could not have received an International Disease Classification, Ninth Revision, code for AA. The cumulative lifetime risk of AA was estimated using the Practical Incidence Estimators method. The hazard ratios (HRs) for mortality, morbidity, and cardiac interventions were compared between those with right- and left-sided lesions after adjustment for age, gender, disease severity, and cardiac risk factors. In a population of 71,467 patients, 7,756 adults developed AAs (isolated right-sided, 2,229; isolated left-sided, 1,725). The lifetime risk of developing AAs was significantly greater in patients with right- sided than in patients with left-sided lesions (61.0% vs 55.4%, p <0.001). The HR for mortality and the development of stroke or heart failure was similar in both groups (HR 0.96, 95% confidence interval [CI] 0.86 to 1.09; HR 0.94, 95% CI 0.80 to 1.09; and HR 1.10, 95% CI 0.98 to 1.23, respectively). However, the rates of cardiac catheterization (HR 0.63, 95% CI 0.55 to 0.72), cardiac surgery (HR 0.40, 95% CI 0.36 to 0.45), and arrhythmia surgery (HR 0.77, 95% CI 0.6 to 0.98) were significantly less for patients with right-sided lesions. In conclusion, patients with right-sided lesions had a greater lifetime burden of AAs. However, their morbidity and mortality were no less than those with left-sided lesions, although the rate of intervention was substantially different.

The cardiac veins in congenitally corrected transposition of the great arteries: delivery options for cardiac devices.

BACKGROUND: Device implantation is sometimes required in congenitally corrected transposition of the great arteries (ccTGA) because of morphologic right ventricular (RV) dysfunction and complete heart block. The anatomical course of the veins remains unknown, despite well-described coronary arterial anatomy. Knowledge of the venous anatomy may facilitate planning of percutaneous cardiac procedures in these patients. OBJECTIVE: This study sought to characterize the venous anatomy in ccTGA. METHODS: Pathologic cardiac specimens from patients with ccTGA were identified from the Mayo Clinic pathology database. Coronary sinus (CS) anatomy and distances from the CS ostium to the major cardiac veins were evaluated. Thebesian veins with ostial openings >1 mm, epicardial veins, and venous collaterals were also quantified. RESULTS: There were 56 hearts with a diagnosis of ccTGA identified. The CS was unidentifiable in 5 hearts due to slicing, and assessment of the Thebesian veins was not possible in 16. Seven hearts had an abnormal CS, 2 of which had atretic ostia and the other 5 of which either had an abnormal ostial location or multiple ostia. There were 28 hearts with at least 1 Thebesian vein with an ostial opening >1 mm. All 12 hearts with unidentifiable Thebesian veins had venous collaterals from the right ventricle (RV) to the major cardiac veins. Epicardial veins extended to the proximal, middle, and distal thirds of the RV in 71%, 23%, and 6%, respectively. CONCLUSION: In ccTGA, the ventricular venous anatomy is abnormal and follows the morphologic RV. However, large interventricular and Thebesian veins may offer options for percutaneous lead or catheter placement when approaching the systemic RV.