Transvenous pacing through the pulmonary valve in a patient with cyanotic congenital heart disease after Glenn shunt - A case report.
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ABSTRACT:
SUBMITTER: Bhyravavajhala S
PROVIDER: S-EPMC7952776 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Complex cyanotic congenital heart diseases with left isomerism are sometimes associated with atrioventricular nodal conduction disturbances that may need permanent pacing. Surgical palliation in such anatomy connecting the superior vena cava to the pulmonary artery precludes a transvenous access for an endocardial pacing lead to the ventricles. Epicardial leads in these patients fail if the pacing thresholds are very high. We report transhepatic permanent ventricular lead implantation for a young boy with heterotaxy complicated by complete heart block.
| S-EPMC5031869 | biostudies-literature
Project description: Not available
| S-EPMC9196930 | biostudies-literature
Project description:Pheochromocytoma and paraganglioma (PHEO-PGL) and cyanotic congenital heart disease (CCHD) are both rare diseases. We reported a 30-year-old patient with a right adrenal gland nodule and a retroperitoneal mass and history of functional single atrium and ventricle. 123I-metaiodobenzylguanidine scintigraphy showed intense uptake in both lesions. Laboratory investigation demonstrated elevated urinary norepinephrine. Preoperative ?-blockade was initiated. A successful open resection of right adrenal and retroperitoneal masses was performed. Pathological examination confirmed PHEO-PGL. Postoperative urinary norepinephrine returned to normal level. A systematic case review in English publications in PubMed and EMBASE suggested a hypothesis that there may exist a possible link between PHEO-PGL and hypoxia from CCHD, which was also indicated in our case. Due to higher risk for PHEO-PGL, a lower threshold of suspicion should be considered in CCHD patients. Therefore, active screening and early treatment of PHEO-PGL are recommended in CCHD patients and clinicians should keep on a long-term follow-up to monitor PHEO-PGL recurrence if hypoxia is not corrected.
| S-EPMC5914282 | biostudies-literature
Project description:ObjectivesSevere hypoxemia in the early postoperative period after bidirectional cavopulmonary shunt (BCPS) is a critical complication. We aimed to evaluate patients who underwent additional systemic to pulmonary shunt and septation of central pulmonary artery (partial takedown) after BCPS.MethodsThe medical records of all patients who underwent BCPS between 2007 and 2020 were reviewed. Patients who underwent partial takedown were extracted and their outcomes were analyzed.ResultsOf 441 BCPS patients, 27 patients (6%) required partial takedown. Most frequent diagnosis was hypoplastic left heart syndrome (n = 14; 52%). Additional complicating factors included pulmonary artery hypoplasia (n = 12) and pulmonary venous obstruction (n = 3). Thirteen patients (48%) underwent partial takedown on the same day of BCPS, and all of them survived the procedure. The remaining 14 patients (52%) underwent partial takedown between postoperative 1 to 64 days. The reasons for partial takedown were: postoperative high pulmonary vascular resistance (n = 4), early BCPS (<90 days) with PA hypoplasia (n = 3), mediastinitis/pneumonia (n = 3), pulmonary venous obstruction (n = 2), ventricular dysfunction (n = 1), and recurrent pneumothorax (n = 1). Four patients experienced hospital deaths. Six patients died after discharge, 10 achieved Fontan completion, and 6 were alive and waiting for Fontan. Overall survival after partial takedown was 54% at 3 years. The pulmonary venous obstruction (P = .041) and genetic/extracardiac anomalies (P = .085) were identified as risks for mortality after partial takedown.ConclusionsThe partial takedown resulted in a 3-year survival rate of more than 50%. Of these patients, a significant number underwent successful Fontan completion who would exhibit potential early death with conservative treatment.
| S-EPMC9510880 | biostudies-literature
Project description:Background:Since oxygen saturation from pulse oximetry (SpO2) and partial pressure of arterial oxygen (PaO2) are observed to improve immediately after surgical correction of cyanotic congenital heart disease (CHD), we postulate that cerebral (CrO2) and somatic (SrO2) oximetry also improves immediately post-correction. We aim to prospectively examine CrO2 and SrO2, before, during, and after surgical correction as well as on hospital discharge in children with cyanotic CHD to determine if and when these variables increase. Methods:This is a prospective observational trial. Eligibility criteria included children below 18?years of age with cyanotic CHD who required any cardiac surgical procedure. CrO2 and SrO2 measurements were summarized at six time-points for comparison: (1) pre-cardiopulmonary bypass (CPB); (2) during CPB; (3) post-CPB; (4) Day 1 in the pediatric intensive care unit (PICU); (5) Day 2 PICU; and (6) discharge. Categorical and continuous variables are presented as counts (percentages) and median (interquartile range), respectively. Results:Twenty-one patients were analyzed. 15 (71.4%) and 6 (28.6%) patients underwent corrective and palliative surgeries, respectively. In the corrective surgery group, SpO2 increased immediately post-CPB compared to pre-CPB [99 (98, 100) vs. 86% (79, 90); p?<?0.001] and remained in the normal range through to hospital discharge. Post-CPB CrO2 did not change from pre-CPB [72.8 (58.8, 79.0) vs. 72.1% (63.0, 78.3); p?=?0.761] and even decreased on hospital discharge [60.5 (53.6, 62.9) vs. 72.1% (63.0, 78.3); p?=?0.005]. Post-CPB SrO2 increased compared to pre-CPB [87.3 (77.2, 89.5) vs. 72.7% (65.6, 77.3); p?=?0.001] but progressively decreased during PICU stay to a value lower than baseline at hospital discharge [66.9 (57.3, 76.9) vs. 72.7% (65.6, 77.3); p?=?0.048]. Conclusion:CrO2 and SrO2 did not increase after corrective surgery of cyanotic CHD even up to hospital discharge. Future larger studies are required to validate these findings. (This study is registered with ClinicalTrials.gov ID: NCT02417259.).
| S-EPMC5908891 | biostudies-literature
Project description:BackgroundLongevity of the superior cavopulmonary connection (SCPC) is limited by the development of pulmonary arteriovenous malformations (PAVM). The goal of this study was to determine whether phenotypic changes in pulmonary artery endothelial cells (PAEC) that favor angiogenesis occur with PAVM formation.MethodsA superior vena cava to right pulmonary artery connection was constructed in 5 pigs. Pulmonary arteries were harvested at 6 to 8 weeks after surgery to establish cultures of PAEC and smooth muscle cells, to determine cell proliferation, gene expression, and tubule formation. Abundance of proteins related to angiogenesis was measured in lung tissue.ResultsContrast echocardiography revealed right-to-left shunting, consistent with PAVM formation. While the proliferation of smooth muscle cells from the right pulmonary artery (shunted side) and left pulmonary artery (nonshunted side) were similar, right PAEC proliferation was significantly higher. Expression profiles of genes encoding cellular signaling proteins were higher in PAECs from the right pulmonary artery versus left pulmonary artery. Protein abundance of angiopoietin-1, and Tie-2 (angiopoietin receptor) were increased in the right lung (both p < 0.05). Tubule formation was increased in endothelial cells from the right pulmonary artery compared with the left pulmonary artery (404 ± 16 versus 199 ± 71 tubules/mm(2), respectively; p < 0.05).ConclusionsThese findings demonstrate that PAVMs developed in a clinically relevant animal model of SCPC concomitantly with differential changes in PAEC proliferative ability and phenotype. Moreover, there was a significant increase in the angiopoietin/Tie-2 complex in the right lung, which may provide novel therapeutic targets to attenuate PAVM formation after a SCPC.
| S-EPMC3934798 | biostudies-literature
Project description:ObjectiveTo determine risk factors affecting time-to-death ≤90 and >90 days in children who underwent a modified Blalock-Taussig shunt (MBTS).MethodsData from a retrospective cohort study were obtained from children aged 0-3 years who experienced MBTS between 2005 and 2016. Time-to-death (prior to Glenn/repair), time-to-alive up until December 2017 without repair, and time-to-progression to Glenn/repair following MBTS were presented using competing risks survival analysis. Demographic, surgical and anesthesia-related factors were recorded. Time-to-death ≤90 days and >90 days was analyzed using multivariate time-dependent Cox regression models to identify independent predictors and presented by adjusted hazard ratios (HR) and 95% confidence intervals (CI).ResultsOf 380 children, 119 died, 122 survived and 139 progressed to Glenn/repair. Time-to-death probability (95% CI) within 90 days was 0.18 (0.14-0.22). Predictors of time-to-death ≤90 days (n = 63) were low weight (<3 kg) (HR 7.6, 95% CI:2.8-20.4), preoperative ventilator support (HR 2.7, 95% CI:1.3-5.6), postoperative shunt thrombosis (HR 5.0, 95% CI:2.4-10.4), bleeding (HR 4.5, 95% CI:2.1-9.4) and renal failure (HR 4.1, 95% CI:1.5-10.9). Predictors of time-to-death >90 days (n = 56) were children diagnosed with pulmonary atresia with ventricular septal defect and single ventricle (compared to tetralogy of fallot) (HR 3.2, 95% CI:1.2-7.7 and HR 3.1, 95% CI:1.3-7.6, respectively), shunt size/weight ratio >1.1 vs <0.65 (HR 6.8, 95% CI:1.4-32.6) and longer duration of mechanical ventilator (HR 1.002, 95% CI:1.001-1.004). Shunt size/weight ratio ≥1.0 (vs <1.0) and ≥0.65 (vs <0.65) were predictors for overall time-to-death in neonates and toddlers, respectively (HR 13.1, 95% CI:2.8-61.4 and HR 7.8, 95% CI:1.7-34.8, respectively).ConclusionsPerioperative factors were associated with time-to-death ≤90 days, whereas particular cardiac defect, larger shunt size/weight ratio, and longer mechanical ventilation were associated with time-to-death >90 days after receiving MBTS. Larger shunt size/weight ratio should be reevaluated within 90 days to minimize the risk of shunt over flow.
| S-EPMC7822344 | biostudies-literature
Project description:Shunt thrombosis remains a major cause of morbidity and mortality, especially during the initial palliation for single-ventricle physiology. The authors present evidence that the P2Y12 inhibitor cangrelor may fill a therapeutic void in thromboprophylaxis. They base this theory on results showing that platelets from neonatal patients with cyanotic congenital heart disease have a robust response to adenosine diphosphate and are amenable to P2Y12 inhibition with cangrelor. Unique to this study was their ability to establish drug efficacy in an avatar mouse model that permits the in vivo evaluation of human platelet-mediated thrombus formation illustrating that this P2Y12 inhibitor yields the intended biological response.
| S-EPMC5646421 | biostudies-literature
Project description:The reversed Potts shunt is designed to offload the right ventricle in severe pulmonary arterial hypertension. We present a case of bidirectional flow across a reversed Potts shunt leading to pulmonary edema, with clinical improvement after implantation of a transcatheter valve in the shunt to maintain unidirectional flow. (Level of Difficulty: Advanced.).
| S-EPMC9706157 | biostudies-literature
Project description:AimsLeft bundle branch area pacing (LBBAP) has been shown to be effective and safe. Limited data are available on LBBAP in the congenital heart disease (CHD) population. This study aims to describe the feasibility and safety of LBBAP in CHD patients compared with non-CHD patients.Methods and resultsThis is a single-centre, non-randomized observational study recruiting consecutive patients with bradycardia indication. Demographic data, ECGs, imaging, and procedural data including lead parameters were recorded. A total of 39 patients were included: CHD group (n = 13) and non-CHD group (n = 26). Congenital heart disease patients were younger (55 ± 14.5 years vs. 73.2 ± 13.1, P < 0.001). Acute success was achieved in all CHD patients and 96% (25/26) of non-CHD patients. No complications were encountered in either group. The procedural time for CHD patients was comparable (96.4 ± 54 vs. 82.1 ± 37.9 min, P = 0.356). Sheath reshaping was required in 7 of 13 CHD patients but only in 1 of 26 non-CHD patients, reflecting the complex and distorted anatomy of the patients in this group. Lead parameters were similar in both groups; R wave (11 ± 7 mV vs. 11.5 ± 7.5, P = 0.881) and pacing threshold (0.6 ± 0.3 V vs. 0.7 ± 0.3, P = 0.392). Baseline QRS duration was longer in the CHD group (150 ± 28.2 vs. 118.6 ± 26.6 ms, P = 0.002). Despite a numerically greater reduction in QRS and a similar left ventricular activation time (65.9 ± 6.2 vs. 67 ± 16.8 ms, P = 0.840), the QRS remained longer in the CHD group (135.5 ± 22.4 vs. 106.9 ± 24.7 ms, P = 0.005).ConclusionLeft bundle branch area pacing is feasible and safe in CHD patients as compared to that in non-CHD patients. Procedural and fluoroscopy times did not differ between both groups. Lead parameters were satisfactory and stable over a short-term follow-up.
| S-EPMC9935007 | biostudies-literature