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Atretic right superior vena cava with left superior vena cava draining directly into left atrium with absent coronary sinus in an adult with partial atrioventricular canal defect and complete heart block.

J Card Surg. 2020 Jul 11;:

Authors: Mishra AK, Jaswal V, Kynta RL, Bansal V, Kumar V, Singh RS

The association of atretic right superior vena cava with persistent left superior vena cava draining directly into left atrium with absent coronary sinus in atrioventricular canal defect is virtually unknown in adults with no case reported so far. Though atretic right superior vena cava with persistent left superior vena cava is an extremely rare venous anomaly seen in congenital heart disease, it has important clinical implications in cardiac surgery and interventional cardiology. Atrial arrhythmias and right bundle branch block are common with advancing age in partial atrioventricualr canal defect but complete heart block has scarcely been reported in the medical literature.

PMID: 32652729 [PubMed - as supplied by publisher]

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Assessment of Disease Progression in Patients With Repaired Tetralogy of Fallot Using Cardiac Magnetic Resonance Imaging: A Systematic Review.

Heart Lung Circ. 2020 Jul 03;:

Authors: Mohamed I, Stamm R, Keenan R, Lowe B, Coffey S

AIMS: Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease with a growing population of adult survivors. Late pulmonary outflow tract and pulmonary valve postoperative complications are frequent, leading to long-term risks such as right heart failure and sudden death secondary to arrhythmias. Cardiac magnetic resonance imaging (CMR) is the gold standard for assessment of cardiac function in patients with repaired ToF. We aimed to determine the most useful CMR predictors of disease progression and the optimal frequency of CMR.
METHODS AND RESULTS: We systematically reviewed PubMed from inception until 29 April 2019 for longitudinal studies assessing the relationship between CMR features and disease progression in repaired ToF. Fourteen (14) studies were identified. Multiple studies showed that impaired right and left ventricular function predict subsequent disease progression. Right ventricular end diastolic volume, while being associated with disease progression when analysed alone, was generally not associated with disease progression on multivariate analysis. Severity of tricuspid regurgitation and pulmonary regurgitant likewise did not show a consistent association with subsequent events. A number of non-CMR factors were also identified as being associated with disease progression, in particular QRS duration and older age at repair. Restrictive right ventricular physiology was not consistently an independent predictor of events.
CONCLUSION: Impaired right and left ventricular function are the most consistent independent predictors of disease progression in repaired ToF. The optimal timing of repeat cardiac imaging remains controversial. Large scale prospective studies will provide important information to guide clinical decision making in this area.

PMID: 32653300 [PubMed - as supplied by publisher]

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"Echo pause" for postoperative transthoracic echocardiographic surveillance.

Echocardiography. 2019 11;36(11):2078-2085

Authors: Cox K, Arunamata A, Krawczeski CD, Reddy C, Kipps AK, Long J, Roth SJ, Axelrod DM, Hanley F, Shin A, Selamet Tierney ES

BACKGROUND: No guidelines exist for inpatient postoperative transthoracic echocardiographic (TTE) surveillance in congenital heart disease. We prospectively evaluated indications for postoperative TTEs in patients with congenital heart disease to identify areas to improve upon (Phase 1) and then assessed the impact of a simple pilot intervention (Phase 2).
METHODS: We included patients with RACHS-1 (Risk Adjustment for Congenital Heart Surgery) scores of 2 and 3 to keep the cohort homogenous. During Phase 1, we collected data prospectively to identify postoperative TTEs for which there were no new findings and no associated clinical management decisions ("potentially redundant" TTEs). During Phase 2, prior to placement of a TTE order, an "Echo Pause" was performed during rounds to prompt review of prior TTE results and indication for the current order. The number of "potentially redundant" TTEs during Phase 1 vs. Phase 2 was compared.
RESULTS: During Phase 1, 98 postoperative TTEs were performed on 51 patients. Potentially "redundant" TTEs were identified in two main areas: (a) TTEs ordered to evaluate pericardial effusion and (b) TTEs ordered with the indication of "postoperative," "follow-up," or "discharge" in the setting of a prior complete postoperative TTE and no apparent change in clinical status. During Phase 2, 101 TTEs were performed on 63 patients. The number of "potentially redundant" TTEs decreased from 14/98 (14%) to 5/101 (5%) (P = .026).
CONCLUSION: Our results suggest that the number of "potentially redundant" TTEs during inpatient postoperative surveillance of patients with congenital heart disease can be decreased by a simple intervention during rounds such as an "Echo Pause."

PMID: 31628768 [PubMed - indexed for MEDLINE]

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Prenatal diagnosis of tetralogy of Fallot associated with pulmonary artery sling: Two case reports.

Echocardiography. 2019 11;36(11):2114-2117

Authors: Li T, Han J, Gao S, Hao X, He Y

Tetralogy of Fallot (TOF) is a common condition accounting for 10%-20% of all fetal cyanotic congenital heart disease cases. Pulmonary artery sling (PAS), or aberrant left pulmonary artery, is a rare congenital cardiovascular malformation. Approximately 58%-83% of PAS is associated with other cardiovascular malformations, TOF being rarest. The diagnosis of PAS is generally incidental or made at autopsy. Cases of prenatal diagnoses of TOF associated with PAS have not yet been reported. Here, we report two cases of TOF associated with PAS diagnosed prenatally in our hospital.

PMID: 31705550 [PubMed - indexed for MEDLINE]

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Risk Factors and Predictors of Cardiac Erosion Discovered from 12 Japanese Patients Who Developed Erosion After Atrial Septal Defect Closure Using Amplatzer Septal Occluder.

Pediatr Cardiol. 2020 Feb;41(2):297-308

Authors: Kitano M, Yazaki S, Sugiyama H, Ohtsuki SI, Tomita H

Cardiac erosion is a rare serious complication following Amplatzer septal occluder (ASO) placement for atrial septal defect. Although multiple risk factors have been found, a useful predictor to prevent it has not been established yet. In 12 patients who developed erosion between 2005 and 2016 in Japan, we retrospectively observed patients' characteristics and transesophageal echocardiography findings immediately before and after ASO placement and at erosion onset. We compared risk factors of erosion, including absent aortic rim, device size/body weight ratio, device shape, or Valsalva sinus wall deformation pressed by either disk-edge, the maximum depth of which from the standard curve of the Valsalva wall was defined as Dent, between the 12 patients and 95 patients with Valsalva sinus wall deformation immediately after placement who did not develop erosion for 5 ± 3 years. Of the 12 patients, nine developed pericardial effusion with eight cardiac tamponade and three aorta-atrium fistula; all were surgically rescued. Surgical findings revealed that erosion in all patients occurred at the right and/or left atrial roof beside the Valsalva in the non-coronary cusp on which the disk-edge seemed to be pressing. The mean Dent immediately after the placement in patients with erosion was significantly deeper than without (2.48 ± 0.32 vs. 1.28 ± 0.38; p < 0.001). There were no differences in the other risk factors between the two groups. Dent is believed to be a useful indicator of erosion development after ASO placement. If Dent is > 2.0 mm, it is desirable to change the size or to replace the device.

PMID: 31732763 [PubMed - indexed for MEDLINE]

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Different CMR Imaging Modalities for Native and Patch-Repaired Right Ventricular Outflow Tract Sizing: Impact on Percutaneous Pulmonary Valve Replacement Planning.

Pediatr Cardiol. 2020 Feb;41(2):382-388

Authors: Ferrari I, Shehu N, Mkrtchyan N, Martinoff S, Eicken A, Stern H, Ewert P, Meierhofer C

Percutaneous pulmonary valve replacement (PPVI) in native or patched right ventricular outflow tract (RVOT) has proven to be feasible. The procedure is highly dependent on the size of the RVOT. Several methods exist to evaluate the size of the RVOT by cardiovascular magnetic resonance (CMR). We evaluated different CMR modalities for measuring RVOT diameters. Thirty-one consecutive patients with native or patched RVOT were retrospectively evaluated. CMR was part of follow-up of patients with corrected Tetralogy of Fallot or pulmonary stenosis with significant pulmonary regurgitation (PR). CMR included 3D-SSFP whole-heart in systole, diastole, and contrast-enhanced MR angiography (ceMRA). Diameters of the RVOT were assessed by the three sequences. Additionally, in patients who underwent cardiac catheterization (n = 11) for PPVI, vessel diameters assessed by cine-angiography were compared to CMR. Systolic diameters of RVOT were significantly larger compared to the diameters taken in diastole and ceMRA (median difference 5.0 mm and 3.8 mm). Diastolic and ceMRA diameters did not differ significantly. CMR diameters taken in systole showed no statistical difference to systolic diameters taken by cine-angiography, while diastolic and ceMRA diameters were significantly smaller. PPVI was feasible to a maximal CMR diameter of 31 mm measured by SSFP whole-heart sequence in systole. Absolute diameters of native RVOT differ depending on the CMR sequence and timing of acquisition (systolic vs diastolic gating). Diameters taken during heart catheterization by cine-angiography best correlate to systolic CMR values. Data may help to select RVOTs suitable for PPVI.

PMID: 31858202 [PubMed - indexed for MEDLINE]

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Management of Persistent Pulmonary Hypertension After Correction of Congenital Heart Defect with Autologous Marrow-Derived Mononuclear Stem Cell Injection into the Pulmonary Artery: A Pilot Study.

Pediatr Cardiol. 2020 Feb;41(2):398-406

Authors: Amoozgar H, Banafi P, Mohammadi H, Edraki MR, Mehdizadegan N, Ajami G, Borzouee M, Keshaarz K, Moradi P, Dehghani E

Pulmonary arterial hypertension (PAH) related to left-to-right shunt can progress to Eisenmenger syndrome, a serious and fatal disease that is not yet curable. This pilot study considered stem cell injection as a new treatment modality in persistent pulmonary hypertension after the correction of a congenital heart defect. Three patients with persistent pulmonary hypertension after ventricular septal defect repair were included in this pilot study for a clinical trial. Mononuclear stem cells derived from patients' bone marrow specimens were injected into the right and left pulmonary arteries via cardiac catheterization. The patients were followed over a 6-month period, with six-minute walk test, echocardiography and repeated angiography performed in the sixth month after treatment. The results of the study showed improvement of 40 m, 280 m and 100 m in 6-minute walk distance in patients 1 to 3, respectively. The peak PR gradient decreased 2, 5 and 9 mmHg by echocardiography, and mean PA pressure decreased 21, 22 and 9 mmHg by catheterization in patients 1 to 3, respectively. Pulmonary artery resistance decreased 4, 4.5 and 1.3 Wood units after 6 months of stem cell therapy in the three patients. No short-term complications were detected in this pilot trial, and all patients tolerated the procedure without any complications. Intrapulmonary artery injection of stem cells may have a role in the treatment of persistent PAH secondary to congenital heart disease. This procedure is feasible, with no significant complications, and this study can be considered as a platform for larger studies.

PMID: 31912176 [PubMed - indexed for MEDLINE]

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An Overview of First 100 Cardiac Surgery Cases at a Newly Developed Satellite Center in Sukkur, Pakistan.

Cureus. 2020 Jun 07;12(6):e8490

Authors: Zia K, Mangi AR, Minhaj S, Tariq K, Rabbi F, Musharaf M, Awan MAB, Memon RA, Rathore AR, Chaudry PA

Introduction The aim of this research is to evaluate the in-hospital and early outcomes of the first 100 adult cardiac surgeries performed at a newly developed satellite center in Sukkur, Pakistan. Methods This is an audit of the first 100 adult cardiac surgeries performed at a newly developed satellite center of the National Institute of Cardiovascular Diseases (NICVD) at Sukkur, Pakistan, from March 2018 to November 2018 with 12 months of post-operative follow-up. Patients were offered off-pump coronary artery bypass (OPCAB), on-pump coronary artery bypass (ONCAB), mitral valve replacement (MVR), aortic valve replacement (AVR), minimally invasive cardiac surgery (MICS), and congenital adult congenital heart disease (ACHD) procedures by expert faculty of NICVD with a minimum of five years of post-fellowship experience. Results The mean age was 47.11 ± 14.6 years, with a male predominance of 77%. Hypertension and smoking were the most common risk factors that were observed in 32% and 33%, respectively, followed by diabetes and dyslipidemia with a frequency of 20% and 9%, respectively. The mean EuroSCORE (European System for Cardiac Operative Risk Evaluation) II for this patient cohort was 1.165 ± 0.50, with a maximum score of 2.3 in one patient. Out of 100 procedures, 51 were ONCAB, 19 were OPCAB, 16 were MVR, three were AVR, nine were ACHD, and two were MICS. Survival status post-operative as well as after one year was 100%. The frequency of post-operative bleeding was 7%, mean post-operative mechanical ventilation time was 213 ± 273 hours, and in-hospital stay was 5.41 ± 0.165 days. Lost to follow-up at one year was 4% (four). During the follow-up assessment, 39.5% of the patients had complained of mild-to-moderate intensity retrosternal pain and 4.2% had superficial surgical site infection of the sternal wound. A significant improvement in functional class was observed in 38.5% of patients, whereas 4.2% (four) had a significant drop in functional class post-operatively. Conclusion Providing tertiary care and early cardiac surgical facility to the people of Sukkur at their doorstep, in a newly developed satellite center, has resulted in improved outcomes, early quality treatment facility, and avoidance of long travel time.

PMID: 32656008 [PubMed]

Pulmonary arterial hypertension: closing the gap in congenital heart disease.

Curr Opin Pulm Med. 2020 Jul 09;:

Authors: Brida M, Nashat H, Gatzoulis MA

PURPOSE OF REVIEW: Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common association adversely affecting quality of life and survival in these patients. We provide herewith recent advances in the understanding and management of PAH-CHD.
RECENT FINDINGS: Significant progress has been made in disease-targeting therapy with pulmonary vasodilators for the treatment of Eisenmenger syndrome, the most severe form of PAH-CHD. Important gaps, however, still exist in the assessment and management of patients with PAH-CHD with systemic to pulmonary shunts. The choice of therapy, either interventional, medical, or both is an on-going dilemma that requires more long-term data. PAH after defect closure represents the most concerning subgroup of patients with the worst prognosis, requiring close follow-up and proactive disease-targeting therapy treatment. Small defects are not considered responsible for patients who have severe PAH and therefore, present different subgroup of patients similar to idiopathic PAH.
SUMMARY: Even with advances in diagnosis and treatment PAH-CHD remains a challenging field requiring lifelong follow-up and meticulous treatment in centres specialized in both CHD and PAH.

PMID: 32657834 [PubMed - as supplied by publisher]

Cardiac Complications during Pregnancy Related to Parity in Women with Congenital Heart Disease.

Cardiology. 2020 Jul 13;:1-9

Authors: Furenäs E, Eriksson P, Wennerholm UB, Dellborg M

OBJECTIVE: To describe the frequency of cardiac complications during pregnancy related to parity in women with congenital heart defects.
METHODS: A retrospective tertiary single-center study at the Adult Congenital Heart Disease Centre that followed 307 women with congenital heart disease during the years 1997-2015 in Gothenburg, Sweden. Ma-ternal cardiac complications were noted for each pregnancy using medical and obstetric records. The CARPREG I and modified WHO (mWHO) risk classifications were used. Twin pregnancies, miscarriages before gestational week 13, and pregnancy terminations were excluded.
RESULTS: Five hundred seventy-one deliveries and 9 late miscarriages were analyzed. The mean parity was 1.74 per woman (range 1-8). Eighty-four (14.6%) maternal cardiac complications were experienced; arrhythmia (5.7%) and heart failure (4.4%) being the most prevalent, and there was 1 maternal death. Heart failure occurred during the first pregnancy in 12 women (3.9%), in the second pregnancy in 8 women (4.3%), and in the third pregnancy in 4 women (7.7%). CARPREG I and mWHO scores were associated with an increased risk of having a cardiac complication, while parity per se was not associated. The OR for having a maternally uneventful second pregnancy if the first pregnancy was without cardiac complications was 5.47 (95% CI 1.76-16.94) after controlling for CARPREG I and mWHO scores.
CONCLUSION: The risk of severe maternal cardiac complications during pregnancy in women with congenital heart disease is low. In this largest analysis to date with a focus on parity in 307 women, the risk classification predicts the maternal outcome more than parity per se. If the first pregnancy is uneventful, the OR is 5.5 for an uneventful second pregnancy if CARPREG I and mWHO scores remain unchanged.

PMID: 32659772 [PubMed - as supplied by publisher]