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Purpose Isolated cor triatriatum sinistrum (CTS) is a heart malformation in which a perforated fibromuscular membrane divides the left atrium into two chambers. When communication between these chambers is restricted, a patient may have signs and symptoms of mitral stenosis. The later stages of pregnancy are associated with tachycardia and increases in intravascular volume. We describe how this altered physiology may affect pregnant women with asymptomatic CTS. We also review the literature relating to pregnancy in patients with CTS.Clinical features A 30-yr-old primigravida, at 40 weeks of gestation with pre-pregnancy diagnosed asymptomatic CTS, was admitted for delivery. She had no cardiac symptoms during pregnancy, and her vaginal delivery under epidural analgesia was uneventful. This cardiac malformation is infrequently described in pregnant women, but a literature review showed that the physiology of late pregnancy with increases in hemodynamic variables may result in cardiac decompensation. Conclusion While our patient with isolated CTS and an unrestrictive intra-atrial membrane had an asymptomatic pregnancy and an uneventful labour, the literature review suggests that the increase in intravascular volume and heart rate that occurs during late pregnancy and after delivery may result in cardiac decompensation, even in patients with asymptomatic CTS. RésuméObjectif Le coeur triatrial gauche (CTG) isole´est une malformation cardiaque dans laquelle une membrane fibromusculaire perfore´e divise l'oreillette gauche en deux chambres. Lorsque la communication entre ces chambres est restreinte, le patient pourrait pre´senter les signes et symptômes d'une ste´nose mitrale. En fin de grossesse, on peut observer de la tachycardie et des augmentations du volume intravasculaire. Nous de´crivons la façon dont cette physiologie modifie´e pourrait affecter les femmes enceintes atteintes de CTG asymptomatique. Nous passons e´galement en revue la litte´rature portant sur la grossesse chez les patientes atteintes de CTG. É léments cliniques Une primigeste de 30 ans, a`40 semaines de grossesse et chez laquelle un diagnostic de CTG asymptomatique avait e´te´pose´avant la grossesse, a e´te´admise a`l'hôpital pour l'accouchement. Pendant la grossesse, elle n'a pre´sente´aucun symptôme cardiaque, et Drs Bojanić and Bursać contributed equally to this work.
Purpose Isolated cor triatriatum sinistrum (CTS) is a heart malformation in which a perforated fibromuscular membrane divides the left atrium into two chambers. When communication between these chambers is restricted, a patient may have signs and symptoms of mitral stenosis. The later stages of pregnancy are associated with tachycardia and increases in intravascular volume. We describe how this altered physiology may affect pregnant women with asymptomatic CTS. We also review the literature relating to pregnancy in patients with CTS.Clinical features A 30-yr-old primigravida, at 40 weeks of gestation with pre-pregnancy diagnosed asymptomatic CTS, was admitted for delivery. She had no cardiac symptoms during pregnancy, and her vaginal delivery under epidural analgesia was uneventful. This cardiac malformation is infrequently described in pregnant women, but a literature review showed that the physiology of late pregnancy with increases in hemodynamic variables may result in cardiac decompensation. Conclusion While our patient with isolated CTS and an unrestrictive intra-atrial membrane had an asymptomatic pregnancy and an uneventful labour, the literature review suggests that the increase in intravascular volume and heart rate that occurs during late pregnancy and after delivery may result in cardiac decompensation, even in patients with asymptomatic CTS. RésuméObjectif Le coeur triatrial gauche (CTG) isole´est une malformation cardiaque dans laquelle une membrane fibromusculaire perfore´e divise l'oreillette gauche en deux chambres. Lorsque la communication entre ces chambres est restreinte, le patient pourrait pre´senter les signes et symptômes d'une ste´nose mitrale. En fin de grossesse, on peut observer de la tachycardie et des augmentations du volume intravasculaire. Nous de´crivons la façon dont cette physiologie modifie´e pourrait affecter les femmes enceintes atteintes de CTG asymptomatique. Nous passons e´galement en revue la litte´rature portant sur la grossesse chez les patientes atteintes de CTG. É léments cliniques Une primigeste de 30 ans, a`40 semaines de grossesse et chez laquelle un diagnostic de CTG asymptomatique avait e´te´pose´avant la grossesse, a e´te´admise a`l'hôpital pour l'accouchement. Pendant la grossesse, elle n'a pre´sente´aucun symptôme cardiaque, et Drs Bojanić and Bursać contributed equally to this work.
BackgroundWe performed a systematic review of cor triatriatum sinistrum (CTS) diagnosed in adults. The aim of this review was to describe the clinical presentation, natural history and management of this congenital heart disease.MethodsA PubMed literature search for ‘cor triatriatum sinistrum’ published since 2005 was performed. Included patients were divided into those with and without obstructive membrane physiology. The clinical course differences were compared.ResultsA total of 171 published cases were included. The median age at diagnosis was 43 years (IQR, 30–60). Obstructive membrane physiology was observed in 70 (41%), and this patient group was younger at presentation (median age 39 (IQR, 28–52) vs 50 years (IQR, 32–64), p=0.003).Patients with obstructive membrane more frequently had associated cardiac defects (58.6% vs 42.4%, p=0.039). Overall, the most frequent clinical symptom was atrial fibrillation, as this was present in 56 (32.8%) of all patients. CTS-related symptoms were more frequent in patients with obstructive membrane: congestive heart failure (44.3% vs 15.2%, p<0.001), pulmonary hypertension (27.1% vs 6.1%, p<0.001), haemorrhage (8.6% vs 0%, p=0.004) and infections manifestation (8.6% vs 0%, p=0.004).A total of 71 (41.5%) patients with CTS required interventional treatment, mainly within patients with the obstructive membrane (86.8% vs 12.6%, p<0.001).ConclusionThe natural history of CTS most often manifests with symptoms of congestive heart failure. Patients with obstructive membrane most often have associated cardiac defects and higher risk for infections and haemorrhage. The interventional treatment of CTS remains the first choice for obstructive membrane.
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