Project description:Takotsubo syndrome or broken-heart syndrome is a rare form of nonischemic cardiomyopathy characterized by regional systolic dysfunction of the left ventricle without evidence of coronary artery disease or acute plaque rupture. This transient impairment in myocardial contractility leads to symptoms and signs that can mimic a myocardial infarction. We present a case of Takotsubo syndrome in a 47-year-old premenopausal woman with complex congenital heart disease who initially presented with acute onset of shortness of breath and chest tightness after a verbal altercation. Extremely rare cases of Takotsubo syndrome have been described in the congenital heart disease population in premenopausal women. This case emphasizes the need to highlight acquired cardiac disease in patients with adult congenital heart disease as this cohort continues to age.
Project description:BackgroundTetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. It is generally diagnosed and surgically repaired early in life, with good overall outcomes.Case presentationWe report the case of a patient incidentally diagnosed with paucisymptomatic TOF at the age of 56 years old, during investigations for carbon monoxide poisoning. The patient had a history of thyroidectomy, arterial hypertension, and four uncomplicated vaginal deliveries.ConclusionsThis case shows us that some patients with TOF can reach older ages without surgical correction. Late surgical repair should be meticulously decided on a case basis.
Project description:BackgroundTetralogy of Fallot (TOF) is one of the most widespread cyanotic congenital heart disease (CHD), which can be successfully repaired in the neonatal period. However, residual problems and the surgical technique itself can create a favourable basis for arrhythmias and conduction abnormalities in these patients. Sometimes, these arrhythmias may worsen during pregnancy and require urgent intervention.Case summaryThis is a case of a 25-year-old woman, who underwent a surgical repair of TOF at the age of 2 years. She suffered an ischaemic stroke postoperatively, which was complicated with secondary seizures and syncope. These episodes were evaluated as epilepsy. The patient was admitted to our hospital with the above-mentioned complaints in the 10th week of pregnancy. A comprehensive cardiac examination was performed. Her presyncopal event was captured during Holter monitoring, which documented a severe dysfunction of the sinus node. She was diagnosed with postoperative sick sinus syndrome and implanted with a permanent dual-chamber pacemaker (PM). After the operation, the patient did not have any episodes of syncope or seizures and the PM check-up showed almost 30% of atrial pacing.ConclusionNo matter how obvious the neurological or other nature of syncope may seem, it is advised to exclude the cardiac origin of syncope, especially in patients with repaired CHD. One of the most common complications after surgery of CHD is rhythm and conduction disturbances. In some of these cases, permanent PM implantation can be unavoidable, even during pregnancy. The implantation of the PM device during pregnancy can be performed safely.
Project description:Tetralogy of Fallot (TOF) is the most common congenital cyanotic heart disease and is characterized by an antero-superior deviation of the infundibular septum with a consequent large malaligned ventricular septal defect (VSD) and a pulmonary and sub-pulmonary (infundibular) stenosis. Surgical repair has been the cornerstone of treatment that is electively performed early in their lives between 3 and 6 months of age. With advancements in transcatheter interventions, the complete percutaneous repair of TOF, a complex disease with multiple treatable lesions, is becoming a conceivable possibility. Here, we report the case of total transcatheter correction of an 18-year-old boy with TOF, performed in two stages. The first stage involved addressing the right ventricular outflow tract (RVOT) obstruction with balloon pulmonary valvuloplasty (BPV) and occluding the conal artery using absolute alcohol and a coil. In the second stage, the VSD was closed with a Multifunctional Occluder (MFO) Konar device 14 -12 mm (Lifetech, China). While surgical treatment remains the gold standard for total correction of TOF, the transcatheter approach can also be considered for selected group of patients who are surgically turned-down.
Project description:ObjectivesTetralogy of Fallot is characterized by anterocephalad deviation of the outlet septum, along with abnormal septoparietal trabeculations, which lead to subpulmonary infundibular stenosis. Archives of retained hearts are an important resource for improving our understanding of congenital heart defects and their morphological variability. This study aims to define variations in aortic override, coronary arterial patterns and ventricular septal defects in tetralogy of Fallot as observed in a morphological archive, highlighting implications for surgical management.MethodsThe Birmingham Children's Hospital archive contains 211 hearts with tetralogy of Fallot, of which 164 were analysed [69 (42.1%) unrepaired and 95 (57.9%) operated specimens]. A detailed morphological and geometric analysis was performed using a rigorous 5-layer review process.ResultsAnomalies were observed in the orifices, origins and course of the coronary arteries: 20 hearts (13.0%) had more than 2 orifices and 3 hearts (1.9%) had a single orifice. In 7 hearts (4.3%), a coronary artery crossed the right ventricular outflow tract. The extent of aortic override ranged from 31.0% to 100% (median of 59.5%). The ventricular septal defect was most often perimembranous (139, 84.8%), but we also found muscular (14, 8.5%), atrioventricular (7, 4.3%) and doubly committed juxta-arterial (2, 1.2%) variants.ConclusionsAnatomical variations are common and can impact surgical management. Anomalous coronary arteries may require a conduit rather than a transannular patch. Variability in aortic override determines the size of patch used to baffle blood to the aorta. The type of ventricular septal defect affects patch closure and the risk of postoperative conduction defects.
Project description:BackgroundCotyledonoid dissecting leiomyoma (CDL) is much less common than typical leiomyoma. Macroscopically, it displays multinodular, exophytic, placenta-like cystic masses and extends into the broad ligament, pelvic cavity, and retroperitoneal space. The seemingly malignant gross appearance of the tumor has perplexed gynecologists and pathologists; microscopically, it has no malignant characteristics, such as atypical cells, a high mitotic index, or tumor necrosis. To date, only a few cases of CDL have been reported. Here, we report a case of CDL, highlighting its gross and histological appearance, and present a review of the literature.Case descriptionA 49-year-old woman presented with a history of progressive constipation of 6 months' duration and a palpable left lower abdominal mass of 1 month's duration. Transvaginal ultrasound revealed a bulky uterus containing 2 subserosal fibroids measuring 9.9 cm × 6.9 cm × 6.3 cm and 8.1 cm × 6.6 cm × 6.8 cm, respectively. An abdominal modified radical hysterectomy and bilateral salpingo-oophorectomy was performed. An intraoperative frozen section showed an angioleiomyoma with edema. However, the postoperative paraffin section confirmed a diagnosis of CDL. No abnormalities were observed at the 6-month follow-up visit.ConclusionsDespite its seemingly malignant gross appearance, CDL, based on its microscopic appearance, is a rare benign tumor and has a favorable prognosis.
Project description:In this study, hemodynamic performance of three novel shunt configurations that are considered for the surgical repair of tetralogy of Fallot (TOF) disease are investigated in detail. Clinical experience suggests that the shunt location, connecting angle, and its diameter can influence the post-operative physiology and the neurodevelopment of the neonatal patient. An experimentally validated second order computational fluid dynamics (CFD) solver and a parametric neonatal diseased great artery model that incorporates the ductus arteriosus (DA) and the full patient-specific circle of Willis (CoW) are employed. Standard truncated resistance CFD boundary conditions are compared with the full cerebral arterial system, which resulted 21, -13, and 37% difference in flow rate at the brachiocephalic, left carotid, and subclavian arteries, respectively. Flow splits at the aortic arch and cerebral arteries are calculated and found to change with shunt configuration significantly for TOF disease. The central direct shunt (direct shunt) has pulmonary flow 5% higher than central oblique shunt (oblique shunt) and 23% higher than modified Blalock Taussig shunt (RPA shunt) while the DA is closed. Maximum wall shear stress (WSS) in the direct shunt configuration is 9 and 60% higher than that of the oblique and RPA shunts, respectively. Patent DA, significantly eliminated the pulmonary flow control function of the shunt repair. These results suggests that, due to the higher flow rates at the pulmonary arteries, the direct shunt, rather than the central oblique, or right pulmonary artery shunts could be preferred by the surgeon. This extended model introduced new hemodynamic performance indices for the cerebral circulation that can correlate with the post-operative neurodevelopment quality of the patient.
Project description:Right ventricular (RV) dysfunction early after tetralogy of Fallot (TOF) increases post-operative morbidity. We investigated associations of circulating biomarkers and socioeconomic factors with early post-operative RV systolic function. Single-center prospective cohort study of infants undergoing TOF repair. Six serologic biomarkers of myocardial fibrosis and wall stress collected at the time of surgery were measured with immunoassay. Geocoding was performed for socioeconomic factors. Multivariate adaptive regression splines (MARS) models identified factors associated with RV function parameters: fractional area change (FAC), global longitudinal strain and strain rate, and free wall strain and strain rate. Seventy-one patients aged 3.5 months (IQR 2.4, 5.2) were included. Galectin-3 was the highest ranked predictor for FAC, global longitudinal strain, and free wall strain, and procollagen type-I carboxy-terminal propeptide (PICP) was the highest ranked predictor for global longitudinal strain rate and free wall strain rate. Several neighborhood characteristics were also highly ranked. Models adjusted R2 ranged from 0.71 to 0.85 (FAC, global longitudinal strain/strain rate), and 0.55-0.57 (RV free wall strain/strain rate). A combination of serologic biomarkers, socioeconomic, and clinical variables explain a significant proportion of the variability in RV function after TOF repair. These factors may inform pre-operative risk-stratification for these patients.