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Journal of the American Heart... Jun 2024Sinus venosus atrial septal defect (SVASD) is a rare congenital cardiac anomaly comprising 5% to 10% of all atrial septal defects. Although surgical closure is the... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Sinus venosus atrial septal defect (SVASD) is a rare congenital cardiac anomaly comprising 5% to 10% of all atrial septal defects. Although surgical closure is the standard treatment for SVASD, data on outcomes have been confined to small cohorts. Thus, we conducted a systematic review of the outcomes of SVASD repair.
METHODS AND RESULTS
The primary outcome was death. Secondary outcomes encompassed atrial fibrillation, sinus node dysfunction, pacemaker insertion, cerebrovascular accident, reoperation, residual septal defect, superior vena cava obstruction, and reimplanted pulmonary vein obstruction. Pooled incidences of outcomes were calculated using a random-effects model. Forty studies involving 1320 patients who underwent SVASD repair were included. The majority were male patients (55.4%), with 88.0% presenting with associated anomalous pulmonary venous connection. The weighted mean age was 18.6±12.5 years, and the overall weighted mean follow-up period was 8.6±10.4 years. The in-hospital mortality rate was 0.24%, with a 30-day mortality rate of 0.5% reported in 780 patients. Incidences of atrial fibrillation, sinus node dysfunction, pacemaker insertion, and cerebrovascular accident over the long-term follow-up were 3.3% (2.18%-4.93%), 6.5% (5.09%-8.2%), 2.23% (1.34%-3.57%), and 2.03% (0.89%-2.46%) respectively. Reoperation occurred in 1.36% (0.68%-2.42%) of surgeries, residual septal defect in 1.34% (0.69%-2.42%), superior vena cava obstruction in 1.76% (1.02%-2.9%), and reimplanted pulmonary vein obstruction in 1.4% (0.7%-2.49%).
CONCLUSIONS
This is the first comprehensive analysis of outcomes following surgical repair of SVASD. The findings affirm the safety and effectiveness of surgery, establishing a reference point for evaluating emerging transcatheter therapies. Safety and efficacy profiles comparable to surgical repair are essential for widespread adoption of transcatheter treatments.
Topics: Humans; Heart Septal Defects, Atrial; Treatment Outcome; Cardiac Surgical Procedures; Postoperative Complications; Male; Adolescent; Young Adult; Female; Child; Hospital Mortality; Adult
PubMed: 38874063
DOI: 10.1161/JAHA.123.033686 -
Frontiers in Cardiovascular Medicine 2024Right ventricular (RV) fibrosis represents both adaptive and maladaptive responses to the overloaded RV condition. Its role in pulmonary hypertension (PH) associated...
Right ventricular fibrosis in adults with uncorrected secundum atrial septal defect and pulmonary hypertension: a cardiovascular magnetic resonance study with late gadolinium enhancement, native T1 and extracellular volume.
INTRODUCTION
Right ventricular (RV) fibrosis represents both adaptive and maladaptive responses to the overloaded RV condition. Its role in pulmonary hypertension (PH) associated with secundum atrial septal defect (ASD), which is the most common adult congenital heart disease (CHD), remains poorly understood.
METHODS
We enrolled 65 participants aged ≥18 years old with uncorrected secundum ASD who had undergone clinically indicated right heart catheterization (RHC), divided into the non-PH group ( = 7), PH group ( = 42), and Eisenmenger syndrome (ES) group ( = 16). We conducted cardiovascular magnetic resonance (CMR) studies with late gadolinium enhancement (LGE) imaging, native T1 mapping, and extracellular volume (ECV) measurement to evaluate the extent and clinical correlates of RV fibrosis.
RESULTS
LGE was present in 94% of the population and 86% of the non-PH group, mostly located at the right ventricular insertion point (RVIP) regions. LGE in the septal and inferior RV region was predominantly observed in the ES group compared to the other groups ( = 0.031 and < 0.001, respectively). The mean LGE scores in the ES and PH groups were significantly higher than those in the non-PH group (3.38 ± 0.96 vs. 2.74 ± 1.04 vs. 1.57 ± 0.79; = 0.001). The ES and PH groups had significantly higher degrees of interstitial RV fibrosis compared to those in the non-PH group, indicated by native T1 (1,199.9 ± 68.9 ms vs. 1,131.4 ± 47.8 ms vs. 1,105.4 ± 44.0 ms; < 0.001) and ECV (43.6 ± 6.6% vs. 39.5 ± 4.9% vs. 39.4 ± 5.8%; = 0.037). Additionally, native T1 significantly correlated with pulmonary vascular resistance ( = 0.708, < 0.001), RV ejection fraction ( = -0.468, < 0.001) and peripheral oxygen saturation ( = -0.410, = 0.001).
CONCLUSION
In patients with uncorrected secundum ASD, RV fibrosis may occur before the development of PH and progressively intensify alongside the progression of PH severity. A higher degree of RV fibrosis, derived from CMR imaging, correlates with worse hemodynamics, RV dysfunction, and poorer clinical conditions.
PubMed: 38873272
DOI: 10.3389/fcvm.2024.1395382 -
Diving and Hyperbaric Medicine Jun 2024We report a compressed air worker who had diffuse cutaneous decompression sickness with pain in his left shoulder and visual disturbance characteristic of migraine aura...
We report a compressed air worker who had diffuse cutaneous decompression sickness with pain in his left shoulder and visual disturbance characteristic of migraine aura after only his third hyperbaric exposure. The maximum pressure was 253 kPa gauge with oxygen decompression using the Swanscombe Oxygen Decompression Table. He was found to have a very large right-to-left shunt across a 9 mm atrial septal defect. He had transcatheter closure of the defect but had some residual shunting with release of a Valsalva manoeuvre. Thirty-two other tunnel workers undertook the same pressure profile and activities in the same working conditions during the maintenance of a tunnel boring machine for a total of 233 similar exposures and were unaffected. As far as we are aware this is the first report of shunt-mediated decompression sickness in a hyperbaric tunnel worker in the United Kingdom and the second case reported worldwide. These cases suggest that shunt-mediated decompression sickness should be considered to be an occupational risk in modern compressed air working. A right-to-left shunt in a compressed air worker should be managed in accordance with established clinical guidance for divers.
Topics: Humans; Decompression Sickness; Male; Heart Septal Defects, Atrial; Occupational Diseases; Compressed Air; Adult; Hyperbaric Oxygenation; Valsalva Maneuver; Middle Aged; Diving
PubMed: 38870955
DOI: 10.28920/dhm54.2.127-132 -
Pediatric Cardiology Jun 2024Hydraulic force aids diastolic filling of the left ventricle (LV) by facilitating basal movement of the atrioventricular plane. The short-axis atrioventricular area...
Hydraulic force aids diastolic filling of the left ventricle (LV) by facilitating basal movement of the atrioventricular plane. The short-axis atrioventricular area difference (AVAD) determines direction and magnitude of this force. Patients with atrial septal defect (ASD) have reduced LV filling due to the left-to-right shunt across the atrial septum and thus potentially altered hydraulic force. The aims were therefore to use cardiac magnetic resonance images to assess whether AVAD and thus the hydraulic force differ in children with ASD compared to healthy children, and if it improves after ASD closure. Twenty-two children with ASD underwent cardiac magnetic resonance before ASD closure. Of these 22 children, 17 of them repeated their examination also after ASD closure. Twelve controls were included. Left atrial and ventricular areas were delineated in short-axis images, and AVAD was defined as the largest ventricular area minus the largest atrial area at each time frame and normalized to body height (AVADi). At end diastole AVADi was positive in all participants, suggesting a force acting towards the atrium assisting the diastolic movement of the atrioventricular plane; however, lower in children both before (6.3 cm/m [5.2-8.0]; p < 0.0001) and after ASD closure (8.7 cm/m [6.6-8.5]; p = 0.0003) compared to controls (12.2 cm/m [11.3-13.9]). Left ventricular diastolic function improves after ASD closure in children by means of improved hydraulic force assessed by AVAD. Although AVADi improved after ASD closure, it was still lower than in controls, indicating diastolic abnormality even after ASD closure. In patients where AVADi is low, ASD closure may help avoid diastolic function deterioration and improve outcome. This could likely be important also in patients with small shunt volumes, especially if they are younger, who currently do not undergo ASD closure. Changes in clinical routine may be considered pending larger outcome studies.
PubMed: 38861174
DOI: 10.1007/s00246-024-03534-5 -
Journal of Gynecology Obstetrics and... Jun 2024To analyze the prenatal and postnatal outcomes of fetuses with d-TGA and to determine whether prenatal echocardiography may predict postnatal urgent BAS.
OBJECTIVE
To analyze the prenatal and postnatal outcomes of fetuses with d-TGA and to determine whether prenatal echocardiography may predict postnatal urgent BAS.
STUDY DESIGN
A retrospective study of fetuses with d-TGA, for which fetal echocardiography was performed at our tertiary hospital from January 2018 to May 2023. We assessed the appearance of the septum primum and the FO flap in the four-chamber view as to whether the FO had a restrictive appearance during measurement of the diameter of the FO at its maximal angle to the attachment point. Color Doppler was used to detect VSDs and measure its diameter both in the four-chamber view and when visualizing the outlets of the great arteries in the sagittal section of the heart.
RESULTS
During the study period, 64 fetuses were diagnosed with d-TGA, which was also confirmed postnatally. Of these, 16 fetuses were excluded due to additional cardiac anomalies or the inability to reach the mother. In total, 48 cases were included in this series. In our study, the FO diameter was significantly decreased in the urgent BAS group, compared with the fetuses without urgent BAS (5.1 mm vs 6.3 mm). A cut off of 6 mm for the FO diameter (sensitivity, 73.3 %; specificity, 72.2 %; area under the curve [AUC], 0.764) and 3.2 mm for the VSD diameter (sensitivity, 75 %; specificity, 75 %; AUC, 0.728) suggested urgent BAS.
CONCLUSION
Prenatal echocardiography performed after 37 weeks of gestation in fetuses with d-TGA provides valuable information to estimate the need for postnatal urgent BAS that would prevent immediate life-threatening complications.
PubMed: 38857825
DOI: 10.1016/j.jogoh.2024.102813 -
The Egyptian Heart Journal : (EHJ) :... Jun 2024The reported prevalence of patent foramen ovale (PFO) in the general population is variable. It ranges between 8.6 and 42% according to the population studied and the...
BACKGROUND
The reported prevalence of patent foramen ovale (PFO) in the general population is variable. It ranges between 8.6 and 42% according to the population studied and the imaging technique used. We aim to prospectively assess the prevalence and characteristics of PFO and interatrial septum (IAS) abnormalities as well as the related clinical manifestations in a sample of Egyptian population.
RESULTS
This study comprised 1000 patients who were referred for CT coronary angiography (CTCA). Mean age was 52.5 ± 10.9 years. The prevalence of PFO among the studied population was 16.3%; closed PFO (grade I) 44.2%, open PFO (grade II) 50.9%, and open PFO with jet (grade III) 4.9%. Anatomical high-risk PFO features-defined as the presence of at least 2 or more of the following (diameter ≥ 2 mm, length ≥ 10 mm, septal aneurysm "ASA", or redundant septum)-were found in 51.5% of PFOs' population. Other IAS abnormalities as redundant septum (8.6%), ASA (5.3%), Bachmann's bundle (4.5%), microaneurysm (2.6%), and atrial septal defect (ASD) (0.4%) were detected. There was a lower rate of coexistence of ASA with PFO (p = 0.031). Syncope was significantly higher in patients with PFO compared to those without PFO (6.7% vs. 1.6%, p = 0.001). Stroke, transient ischaemic attacks (TIA), and dizziness were similar in both groups. TIA, dizziness, and syncope were significantly higher in patients with IAS abnormalities including PFO compared to those without IAS abnormalities. Syncope was also significantly higher in PFO with high-risk anatomical features compared to those with non-high-risk PFO population (p = 0.02).
CONCLUSION
The prevalence of PFO in our study was approximately 16.3%, almost half of them showed anatomical high-risk features for stroke. Dizziness, syncope and TIA were significantly higher in patients with IAS abnormalities including PFO.
PubMed: 38856789
DOI: 10.1186/s43044-024-00504-3 -
Echocardiography (Mount Kisco, N.Y.) Jun 2024Balloon sizing (BS) has been used for device size selection in percutaneous atrial septal defect (ASD) closure. Due to its limitations, alternative imaging techniques... (Comparative Study)
Comparative Study
BACKGROUND
Balloon sizing (BS) has been used for device size selection in percutaneous atrial septal defect (ASD) closure. Due to its limitations, alternative imaging techniques like three-dimensional transesophageal echocardiography (3D-TEE) are valuable for guiding ASD device size selection during ASD closure procedures. The purpose of this study was to compare ASD sizing using measurements obtained from 3D-TEE to those utilizing the standard balloon sizing method.
METHODS
We identified 53 patients with single secundum type ASD without PFO who underwent percutaneous closure at the Tehran Heart Center between 2019 and 2022. Balloon sizing was performed in all patients with the stop-flow technique, and the choice of device size was determined based on the sizing derived from BS. 3D-TEE imaging was performed before the intervention, and the ASD shape and quality of ASD rims were assessed.
RESULTS
Among the 53 patients who underwent single ASD device closure, multiple 3D TEE measurements significantly correlated with balloon sizing results. This included defect area, perimeter, and diameter obtained from 3D-TEE images multi-planar reconstruction. ASD perimeter detected by 3D TEE had the best correlation with BS results. When divided by the shape of ASD, there was no significant difference between our 3D-images data and BS in round or oval-shaped ASDs.
CONCLUSION
The 3D-TEE study is reliable for assessing ASD configurational characteristics in percutaneous device closure candidates. 3D-TEE has the potential to accurately determine the appropriate device size and reduce complications, costs, and procedural duration. Further research is needed to validate these findings and establish the role of 3D-TEE measurements in guiding the best treatment decisions for ASD closure.
Topics: Humans; Echocardiography, Transesophageal; Echocardiography, Three-Dimensional; Male; Heart Septal Defects, Atrial; Female; Septal Occluder Device; Adult; Middle Aged; Adolescent; Prosthesis Design; Cardiac Catheterization; Reproducibility of Results; Young Adult
PubMed: 38853621
DOI: 10.1111/echo.15822 -
BMC Cardiovascular Disorders Jun 2024Atrial septal defects (ASD) are the most common type of adult congenital heart disease (ACHD) associated with a high risk developing of pulmonary arterial hypertension...
BACKGROUND
Atrial septal defects (ASD) are the most common type of adult congenital heart disease (ACHD) associated with a high risk developing of pulmonary arterial hypertension (PAH). ASD closure is not recommended in patients with PAH and Pulmonary Vascular Resistance (PVR) ≥ 5 Wood Unit (WU). Noninvasive methods have been proposed to measure PVR; however, their accuracy remains low. Right Ventricle (RV) - Pulmonary Artery (PA) coupling is defined as the ability of the RV to adapt to high-resistance conditions. Tricuspid Annular Plane Systolic Excursion (TAPSE)/estimated pulmonary artery systolic pressure (ePASP) calculation using echocardiography is a noninvasive technique that has been proposed as a surrogate equation to evaluate RV-PA coupling. Currently, no research has demonstrated a relationship between RV-PA coupling and PVR in patients with ASD.
METHODS
The study participants were consecutive eligible patients with ASD who underwent right heart catheterization (RHC) and echocardiography at Hasan Sadikin General Hospital, Bandung. Both the procedures were performed on the same day. RV-PA Coupling, defined as TAPSE/ePASP > 0.31, was assessed using echocardiography. The PVR was calculated during RHC using the indirect Fick method.
RESULTS
There were 58 patients with ASD underwent RHC and echocardiography. Among them, 18 had RV/PA Coupling and 40 had RV/PA Uncoupling. The PVR values were significantly different between the two groups (p = 0.000). Correlation test between TAPSE/ePASP with PVR showed moderate negative correlation (r= -0.502, p = 0.001). TAPSE/ePASP ≤ 0.34 is the cutoff point to predict PVR > 5 WU with sensitivity of 91.7% and specificity 63.6%.
CONCLUSION
This study showed a moderate negative correlation between TAPSE/ePASP and PVR. TAPSE/ePASP ≤ 0.34 could predict PVR > 5 WU with good sensitivity.
Topics: Humans; Heart Septal Defects, Atrial; Pulmonary Artery; Male; Vascular Resistance; Female; Ventricular Function, Right; Adult; Middle Aged; Cardiac Catheterization; Arterial Pressure; Pulmonary Arterial Hypertension; Predictive Value of Tests
PubMed: 38853261
DOI: 10.1186/s12872-024-03935-7 -
Journal of Veterinary Cardiology : the... May 2024Double outlet right atrium is a rare congenital cardiac abnormality that has been previously reported in humans and cats, but not in dogs. A double outlet right atrium...
Double outlet right atrium is a rare congenital cardiac abnormality that has been previously reported in humans and cats, but not in dogs. A double outlet right atrium is typically characterized by the presence of a leftward deviation of the interatrial septum and atrial septal defect. Therefore, the right atrium drains into both ventricles. The unique features consistent with double outlet right atrium were identified by transthoracic echocardiography and computed tomography in a puppy. This case report describes the clinical, echocardiographic, and tomographic findings of a five-month-old Cocker Spaniel diagnosed with this rare congenital abnormality.
PubMed: 38851121
DOI: 10.1016/j.jvc.2024.05.001 -
European Journal of Case Reports in... 2024Alagille syndrome (ALGS) is a multisystem disorder involving at least three systems among the liver, heart, skeleton, face, and eyes. Common cardiac associations include...
BACKGROUND
Alagille syndrome (ALGS) is a multisystem disorder involving at least three systems among the liver, heart, skeleton, face, and eyes. Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF). Coarctation of aorta (CoA), renal and intracranial arteries are commonly involved vessels in Alagille syndrome. We present two cases with rare cardiovascular manifestations of Alagille syndrome. .
CASE 1
A 25-year-old female with a history of Alagille syndrome presented to the cardiologist office for progressive exertional dyspnoea, orthopnoea, and palpitations. She was tachycardiac on examination and had an apical diastolic rumble. A transthoracic echocardiogram (TTE) showed a left ventricular ejection fraction (LVEF) of 60% and parachute mitral valve (PMV) with severe mitral stenosis. A transoesophageal echocardiogram (TOE) showed insertion of chordae into the anterolateral papillary muscle, severe mitral stenosis with a valve area of 0.7 cm. She was referred to a congenital heart disease specialist and underwent robotic mitral valve replacement with improvement in her symptoms.
CASE 2
A 27-year-old female with known Alagille syndrome and resistant hypertension presented to the cardiologist office due to progressive exertional dyspnoea for a year. She was hypertensive and had a new 2/6 systolic ejection murmur along the left upper sternal border. TTE revealed an LVEF of 60% and pulmonary artery pressure of 19 mmHg. A CoA was suspected distal to the left subclavian artery due to a peak gradient of 38 mmHg. Cardiac magnetic resonance (CMR) imaging ruled out CoA, and diffuse narrowing of the descending thoracic aorta measuring 13-14 mm in diameter was noted. The patient was referred to a congenital heart disease specialist for further management.
CONCLUSION
PMV presenting as mitral stenosis and mid-aortic syndrome are not commonly described anomalies in association with Alagille syndrome. TTE, TOE and CMR played a key role in diagnosis and management of these patients.
LEARNING POINTS
Alagille syndrome (ALGS) is a complex multisystem disorder involving the liver, heart, skeleton, face, and eyes. Cardiovascular involvement occurs in up to 95% of the patients.Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF).A parachute mitral valve (PMV) presenting as mitral stenosis and mid-aortic syndrome is not commonly described anomalies in association with ALGS. Here, we present such rare cases.
PubMed: 38846669
DOI: 10.12890/2024_004545