A Case of Secondary Pulmonary Hypertension in a Patient With Atrial Septal Defect and Fetal Alcohol Syndrome.

We report a case of a 34-year-old man with fetal alcohol syndrome (FAS) presenting with dyspnea, cough, and hoarse voice. The patient was found to have severe pulmonary hypertension secondary to a large atrial septal defect (ASD). In this article, we discuss the challenges patients with FAS and other patients with cognitive impairments face that could explain the first diagnosis of such a large cardiac birth defect being made in the patient's adulthood. Moreover, severe pulmonary hypertension due to ASD also presents a therapeutic dilemma, as shunt closure can lead to a worsening of the condition.

Atrial Septal Defect (ASD) Repair Unveiling an Unusual Conduction Conundrum: A Wenckebach Case Report.

Atrial septal defects are a common congenital malformation that can lead to an elevated risk for stroke due to the bypass of the lungs by deep vein thrombosis, as well as the expected repercussions of pulmonary hypertension if left untreated. Surgical intervention is definitive; however, recent advancements in treatment options, such as percutaneous intervention, represent a safer and equally effective way to treat this congenital complication. While safer, percutaneous interventions can also lead to adverse events that may force patients to present to the emergency department. Here, we present a unique case of a patient with congenital atrial septal defect status post-percutaneous intervention who developed a new-onset second-degree AV block, Mobitz type 1 Wenckebach rhythm.

Atrial Septal Defect vs Ventricular Septal Defect: Getting the Right Mix in Transposition of the Great Arteries.

Transposition of the great arteries (TGA) is the second most common cyanotic congenital cardiac defect and affects around 4.7 in 10,000 live births. Patients present at birth with profound cyanosis due to inadequate oxygen delivery to the systemic circulation. Typical management after birth involves the administration of prostaglandins and oxygen while awaiting surgical repair. Balloon atrial septostomy may be performed depending on the adequacy of the interatrial communication. In this case report, we present a challenging case of TGA ventricular septal defect (VSD) and pulmonary stenosis (PS), demonstrating the importance of bedside clinical examination along with applying basic management principles. The patient underwent a right modified Blalock-Taussig-Thomas shunt (BTT) along with left pulmonary artery (LPA) reconstruction and main pulmonary artery band as an initial palliative procedure. The patient deteriorated post-operatively, with increasing desaturations and oxygen requirements. Though imaging suggested sufficient inter-circulatory mixing, the clinical picture of desaturation without respiratory distress and lack of response to oxygen and pulmonary vasodilatory therapy strongly suggested otherwise. The child therefore underwent a balloon atrial septostomy. Their clinical condition improved and they were discharged three days later. We describe this case's clinical course, medical and surgical management, and learning points.

Successful Atrial Septal Defect Closure Subsequent to Medical Pulmonary Preconditioning in an Infant With Severe Pulmonary Hypertension Associated With Bronchopulmonary Dysplasia.

While atrial septal defect (ASD) may contribute to right ventricular decompression in patients with severe pulmonary hypertension (PH), the pulmonary vasculature might be compromised by increased pulmonary blood flow, even though pulmonary vasodilators successfully reduce resistance. ASD closure is a treatment option that may ameliorate PH symptoms associated with bronchopulmonary dysplasia (BPD) in infants. However, the feasibility of ASD closure is obscure in patients with BPD-PH causing right-to-left shunting. Here, we present an eight-month-old girl with ASD complicated by BPD-PH, in which the pulmonary pressure exceeded the systemic pressure; the ASD was successfully closed after pulmonary preconditioning with dexamethasone and high-dose diuretics. Our patient was delivered as the third baby in triplets at a gestational age of 25 weeks, with a birth weight of 344 g. She was diagnosed with BPD at three months of age (37 weeks of postmenstrual age) with a body weight of 1.4 kg. Mild pulmonary hypertension was identified at the age of five months, and oral sildenafil was initiated. While her atrial septal defect was small at the time of PH diagnosis, it became hemodynamically significant when she grew up to 3.4 kg of body weight, at seven months after birth. Her estimated right ventricular pressure was apparently more than the systemic pressure, and oxygen saturation fluctuated between 82% and 97% under oxygen supplementation due to bidirectional interatrial shunt with predominant right-to-left shunting. Pulmonary preconditioning lowered the estimated right ventricular pressure to almost equal the systemic pressure and elevated arterial oxygen saturation while also suppressing right-to-left shunting. Cardiac catheterization after preconditioning revealed a ratio of pulmonary blood pressure to systemic blood pressure ratio (Pp/Ps) of 0.9, pulmonary resistance of 7.3 WU-m2, and a pulmonary to systemic blood flow ratio (Qp/Qs) of 1.3 (approximately 1.0 in the normal circulation without significant shunt), with the cardiac index of 2.8 L/min/m2. The acute pulmonary vasoreactivity test against the combination of 20 ppm nitric oxide and 100% oxygen was negative, although the patient had consistently high pulmonary flow with makeshift improvements after preconditioning. Despite the high pulmonary resistance even after preconditioning, aggressive ASD closure was performed so that pulmonary flow could be consistently suppressed regardless of the pulmonary condition. Her Pp/Ps under 100% oxygen with 20 ppm nitric oxide was 0.7 immediately after closure. After two years of follow-up, her estimated right ventricular pressure was less than half of the systemic pressure with the use of three pulmonary vasodilators, including sildenafil, macitentan, and beraprost. A strategy to temporarily improve PH and respiratory status aimed at ASD closure could be a treatment option for the effective use of multiple pulmonary vasodilators, by which intensive treatment of BPD can be achieved.

Patients with volume-loaded right ventricle - quantification of left ventricular hemodynamic response to intervention measured by noninvasive pressure-volume loops.

Volume loading of the right ventricle (RV) in patients with atrial septal defect (ASD) and patients with repaired Tetralogy of Fallot (rToF) affects the pumping mechanics of the left ventricle (LV). Intervention of the lesion will relieve the RV volume load however quantifiable impact on exercise capacity, arrhytmias or death are limited. A possible explanation could be remaining effects on the function of the LV. The aim of this study was therefore to investigate if hemodynamics of the LV differs between patients with RV volume load due to ASD or rToF and healthy controls and if they change after intervention. Eighteen patients with ASD, 17 patients with rToF and 16 healthy controls underwent cardiac magnetic resonance imaging (CMR) and maximal exercise test with continuous gas analysis. Reexamination was performed 13 ± 2 months after closure of the ASD in 13 of the patients and 10 ± 4 months after pulmonary valve replacement (PVR) in 9 of the patients with rToF. Non-invasive PV-loops from CMR and brachial pressures were analyzed. Stroke work (SW) and potential energy (PE) increased after ASD closure but not in ToF patients after valve repair. Patients with ASD or rToF had higher contractility and arterial elastance than controls. No major effects were seen in LV energetics or in peak VO2 after ASD closure or PVR. Peak VO2 correlated positively with SW and PE in patients with ASD (r = 0.54, p < 0.05; r = 0.61, p < 0.01) and controls (r = 0.72, p < 0.01; r = 0.53, p < 0.05) to approximately the same degree as peak VO2 and end-diastolic volume (EDV) or end-systolic volume (ESV). In ToF patients there was no correlation between PV loop parameters and peak VO2 even if correlation was found between peak VO2 and EDV or ESV. In conclusion, the LV seems to adapt its pumping according to anatomic circumstances without losing efficiency, however there are indications of persistent vascular dysfunction, expressed as high arterial elastance, which might have impact on exercise performance and prognosis. Future studies might elucidate if the duration of RV volume load and decreased LV filling have any impact on the ability of the vascular function to normalize after ASD closure or PVR.

A Case Series on Successful Surgical Management of Patients Presenting With Lutembacher's Syndrome to a Tertiary Healthcare Center in Northern India.

Atrial septal defect (ASD), whether congenital or iatrogenic, and mitral stenosis (MS), whether congenital or acquired, may all come together into a condition called Lutembacher's syndrome (LS). The ASD is typically larger than 15 mm in a normal LS case. However, congenital ASD is less frequent than residual iatrogenic ASD in the current era of percutaneous balloon mitral valvuloplasty for acquired MS. This is referred to as iatrogenic LS by cardiologists. Hereby, we report a case series of three patients who presented to us, were diagnosed with LS, a very rare entity, and were managed successfully.

Mid-Term Electrical Remodeling after Percutaneous Atrial Septal Defect Closure with GCO Device in a Pediatric Population.

BACKGROUND AND AIM: The GORE® CARDIOFORM (GCO) septal occluder is an atrial septal defect/patent foramen ovale closure device with theoretical advantages over other commercialized devices thanks to its softness and anatomical compliance. Our aim was to evaluate the short- and medium-term electrocardiographic changes after percutaneous ASD closure with GCO in a pediatric population. METHODS: We enrolled 39 patients with isolated ASD submitted to trans-catheter closure from January 2020 to June 2021. ECG was performed before, at 24 h and 6 months after the procedure. P wave dispersion, QTc and QTc dispersion were calculated. ECG Holter was recorded at 6 months after implantation. RESULTS: Patients' age and body surface area (BSA) were 8.2 ± 4.2 years and 1.0 ± 0.3 m2 respectively. At the baseline, mean P wave dispersion was 40 ± 15 msec and decreased at 24 h (p < 0.002), without any further change at 6 months. At 24 h, PR conduction and QTc dispersion significantly improved (p = 0.018 and p < 0.02 respectively), while the absolute QTc value considerably improved after 6 months. During mid-term follow-up, QTc dispersion remained stable without a significant change in PR conduction. The baseline cardiac frequency was 88.6 ± 12.6 bpm, followed by a slight reduction at 24 h, with a further amelioration at 6 months after the procedure (87.3 ± 14.2, p = 0.9 and 81.0 ± 12.7, p = 0.009, respectively). After device deployment, two patients developed transient, self-limited junctional rhythm. One of them needed a short course of Flecainide for atrial ectopic tachycardia. No tachy/brady-arrhythmias were recorded at the 6-month follow-up. ASD closure resulted in a marked decrease in right heart volumes and diameters at 6 months after percutaneous closure. CONCLUSIONS: Percutaneous ASD closure with the GCO device results in significant, sudden improvement of intra-atrial, atrio-ventricular and intraventricular electrical homogeneity. This benefit persists unaltered over a medium-term follow-up. These electrical changes are associated with a documented positive right heart volumetric remodeling at mid-term follow-up.

Common Consequences of Uncommon Congenital Heart Disease: Exploring the Trilogy of Fallot With Infective Endocarditis and Cerebral Venous Thrombosis.

Trilogy of Fallot (ToF) is a rare congenital heart disease characterized by a combination of atrial septal defect, pulmonary stenosis, and right ventricular hypertrophy. It is more common in females and can cause symptoms such as cyanosis and breathlessness. ToF can lead to complications like thromboembolic events, including infective endocarditis (IE) and cerebral venous thrombosis (CVT). This case study discusses a nine-year-old female with ToF who also had IE and CVT. The patient recovered well following treatment with intravenous beta-lactam and aminoglycoside for IE and subcutaneous low-molecular-weight heparin for CVT.

Congenital Atrial Septal Defect Presenting With Tamponade Physiology and an Associated Viral Illness.

Cardiac tamponade is a serious clinical syndrome that often presents with the classic triad of hypotension, jugular vein distention and diminished or muffled heart sounds on auscultation (Beck's Triad). This phenomenon occurs due to fluid accumulation in the pericardial space which compresses the heart, reduces cardiac output and may cause cardiogenic shock. In this report, we present a case of a 22-year-old female with a congenital atrial septal defect (ASD) and right ventricular failure with tamponade physiology with an associated viral illness.

Trans-jugular transcutaneous closure of atrial septal defects with an adjustable curved sheath under echocardiography guidance.

INTRODUCTION AND OBJECTIVES: Traditional transcatheter closure of atrial septal defect (ASD) via the femoral vein carries risk of radiation damage. Transcutaneous closure of ASD under echocardiography guidance avoids radiation exposure and can be gradually applied. An alternative is to transcutaneous closure of ASD trans-jugular with an adjustable curved sheath under echocardiography guidance. METHODS: We retrospectively studied all cases of trans-jugular transcutaneous closure of ASD with an adjustable curved sheath under echocardiography guidance in the Heart Center of Henan Province People's Hospital between 2016 and December 2022. RESULTS: A total of 156 patients were included, 74 males and 82 females. Mean age was 6.9 ± 7.4 years and weight 23.7 ± 14.6 kg. Mean sizes of the ASD and occluder were (9.7 ± 4.7) mm and (14.1 ± 5.7) mm. The mean operation time was (49.6 ± 29.2) min. No complications such as atrioventricular block, reoperation, or pericardial effusion occurred. There are 3 patients had a residual shunt. All patients were followed-up for (38.7 ± 11.0) months. The three patients with residual shunt had self-closed at the 3-6-12 months follow-up. There was no complication at follow-up. CONCLUSION: Trans-jugular transcutaneous closure of ASD with adjustable curved sheath under echocardiography guidance is safe, effective and minimally invasive.

Three-Dimensional Cardiac Imaging: A Necessity to the Diagnosis and Treatment of Swiss-Cheese Atrial Septal Defect.

Swiss-cheese atrial septal defect (ASD) is a malformation characterized by multi-fenestrated interatrial defects. Here, we describe a vignette of a 23-year-old man with Swiss-Cheese ASD characterized by two defects with areas of 0.74 cm2 and 0.44 cm2, complicated with an atrial septal aneurysm successfully repaired with a cribriform amplatzer septal occluder (ASO) via the percutaneous transcatheter approach. This case emphasizes the importance of attaining a clear view of three-dimensional structures for proper device selection and deployment in repair as additional structural defects such as concomitant aneurysms impose significant challenges.

Cleft Palate in a Newborn With Trisomy 21: A Case Report.

Trisomy 21, or Down syndrome (DS), is neonates' most common chromosomal abnormality. In addition, children born with DS have an increased risk of congenital anomalies such as congenital heart defects, gastrointestinal abnormalities, and, rarely, cleft palate. Cleft lip and palate are among the most common congenital anomalies associated with many congenital syndromes; however, Trisomy 21 is the least common congenital anomaly associated with orofacial clefts. We present a case of cleft palate, duodenal stenosis, persistent pulmonary hypertension of the newborn, patent ductus arteriosus, and atrial septal defect in a newborn with classical clinical features of Down syndrome. This report discusses the uncommon presentation of trisomy 21 and concomitant cleft palate in a neonate, including its recognition and treatment, as no standard of care treatment exists.

Migrated Inferior Vena Cava (IVC) Filter Presenting as Tricuspid Valve Mass, Right-Sided Heart Failure, and Parodoxical Emboli.

A 58-year-old male with an unknown medical history presented with acute encephalopathy, receptive aphasia, and hypertensive emergency. The patient did not have any family members from whom a collateral history could be obtained. He underwent X-rays of the abdomen and bilateral humeri/femurs to check for foreign bodies. He was found to have right femoral open reduction and internal fixation with retained screw fragments. He was diagnosed with ischemic stroke on MRI. Transthoracic echocardiogram (TTE) revealed right-sided heart failure and a tricuspid valve mass as well as right to left shunting. This raised concern for large atrial septal defect (ASD) with paradoxical embolization from tricuspid valve mass. Transesophageal echocardiogram (TEE) redemonstrated large ASD. Concern was raised for the ASD closure device as the cause of this "tricuspid mass." Due to history of orthopedic procedure, it was hypothesized that the patient had an IVC filter placed in the setting of pulmonary embolism (PE) prior to an orthopedic procedure. The tricuspid valve was visualized under fluoroscopy and was confirmed to be a migrated IVC filter. He was taken to the operating room (OR) for cardiac surgery for the removal of the IVC filter and repair of ASD. Surprisingly, no ASD was found.

Minimal-Access Atrial Septal Defect (ASD) Closure.

Progress towards the development and adoption of minimally invasive techniques in cardiac surgery has been slower than that seen in other surgical specialties. Congenital heart disease (CHD) patients represent an important population within cardiac disease, of which atrial septal defect (ASD) is one of the most common diagnoses. Management of ASD encompasses a range of minimal-access and minimally invasive approaches, including transcatheter device closure, mini-sternotomy, thoracotomy, video-assisted, endoscopic, and robotic approaches. In this article, we will discuss the pathophysiology of ASD, along with diagnosis, management, and indications for intervention. We will review the current evidence supporting minimally invasive and minimal-access surgical ASD closure in the adult and paediatric patient, highlighting peri-operative considerations and areas for further research.

[Ischemic stroke associated with patent foramen ovale: hospital database analysis]. / Ishemicheskii insul't, assotsiirovannyi s otkrytym oval'nym oknom: analiz dannykh gospital'nykh registrov.

OBJECTIVE: To analyze clinical and instrumental characteristics of patients with ischemic stroke (IS) due to paradoxical embolism according to the data of hospital registers of Moscow and Perm. MATERIAL AND METHODS: A comprehensive study of 114 patients, aged 18 to 55 years, with IS by the mechanism of paradoxical embolism was carried out. All patients underwent clarification of the cause of IS (electrocardiography, ultrasound scanning of the brachiocephalic arteries, CT or MRI of the brain, CT or MR angiography, transthoracic and/or transesophageal echocardiography). The presence of right-left shunt blood flow (RLS) was confirmed by transcranial dopplerography with a bubble test. The clinical significance of patent foramen ovale (PFO) was assessed according to The PFO-Associated Stroke Causal Likelihood Classification System (PASCAL). RESULTS: Clinical and instrumental characteristics of patients with IS due to paradoxical embolism were obtained from two hospital registries. In both groups, the leading trigger for the development of IS was the Valsalva phenomen (>20%), the share of other provoking factors did not exceed 10%. Significant differences between the analyzed groups related to the ultrasonic characteristics of RLS/PFO: patients with a grade 4 shunt predominated in the Research Center of Neurology (RCN) population, while patients with a grade 3 shunt predominated in the City Clinical Hospital (CCH) №4 group. At the same time, there were twice as many patients with aneurysm of the interatrial septum in the CCH №4 group. In accordance with the PASCAL classification, in 93% of RCN patients, PFO can be considered as a probable cause of IS, while in the CCH No. 4 group, a probable causal relationship was traced only in 51% of cases, in 18% of patients, the role of an anomaly in the development of stroke was unlikely. CONCLUSION: The analysis showed that the primary screening of RLS in a regional vascular center allows classifying PFO as a probable cause of IS in only half of the patients. For a more accurate selection of patients for whom endovascular occlusion of the PFO will be most effective, an in-depth examination in a specialized hospital is recommended.

A Rare Case of Severe Isolated Right Heart Failure with Secundum Type Atrial Septal Defect and Mitral Regurgitation Without Pulmonary Hypertension.

Typically, right heart failure (RHF) may occur following left heart failure (LHF) in chronic volume overload states such as chronic severe mitral regurgitation (MR) through chronically elevated pulmonary pressures. In Lutembacher syndrome (LS), the direct shunting through a secundum type atrial septal defect (ASD) results in congestive heart failure in the setting of severe mitral stenosis (MS) with or without elevated pulmonary arterial or venous pressures. We report a rare case of severe isolated RHF and bi-atrial enlargement resulting from the direct shunting through a secundum type ASD in the presence of a severe eccentric primary MR. There are no significant cases documented like this after a thorough search using PubMed, Medline, and Google Scholar. A review of the literature suggests that LS is also caused by a combination of mitral regurgitation and a secundum-type atrial septal defect without mitral stenosis, though rarely. Because this is a primary MR, we feel it is a case of LS with MR, ruling out a combination of secondary MR and secundum-type atrial septal defect.

Recurrent peritonitis relapse in a patient with atrial septal defect undergoing peritoneal dialysis: a case report.

BACKGROUND: Peritonitis is the most common complication in patients undergoing peritoneal dialysis (PD). Most patients recover with appropriate antibiotic treatment; however, when peritonitis repeatedly relapses, the cause of recurrence must be explored. The relationship between atrial septal defect (ASD), infective endocarditis (IE), and peritonitis is rarely reported. Here, we present a case of recurrent peritonitis due to Staphylococcus aureus in a patient with ASD and IE undergoing PD. CASE PRESENTATION: A 46-year-old woman with chronic renal failure secondary to chronic glomerulonephritis experienced three episodes of peritonitis within 80 days of starting PD. The patient had a history of untreated ASD without symptoms. After undergoing PD for approximately 35 days, the patient was admitted to our hospital on April 5, 2016, due to abdominal pain and fever for 1 week (maximum temperature of 38.5 °C) accompanied by chills and shivering. The PD effluent from the time of her admission was positive for S. aureus. Thereafter, peritonitis recurred each month. When the third episode of peritonitis occurred, transthoracic echocardiography was performed, and a vegetation measuring 9.5 × 6.4 mm attached to the surface of the right ventricle around the ventricular septal membrane was identified. Finally, the patient was diagnosed with IE. Then, ASD repair surgery was successfully performed after the infection was controlled. The patient was followed up for 5 years, with no further episodes of recurrence. CONCLUSIONS: When a patient with ASD undergoing PD develops peritonitis, especially relapsing peritonitis, the possibility of IE is significantly increased. ASD repair surgery may be an important contributing factor to prevent peritonitis recurrence.

Operability of atrial septal defect with borderline pulmonary vascular resistance index: A study in developing country.

Background: Pulmonary arterial hypertension secondary to atrial septal defect (ASD) is an important determinant of morbidity and mortality in defect closure. We aimed to compare perioperative outcome between preoperative borderline and low pulmonary vascular resistance index (≥4 WU.m2 and <4 WU.m2, respectively) in surgical closure of secundum atrial septal defect with concomitant pulmonary arterial hypertension. Methods and results: This was a single-center retrospective cohort study between January 2015 and January 2020. We classified patients with low and borderline PVRI who underwent ASD closure and recorded the perioperative outcomes. Results: We analyzed a total of 183 patients with atrial septal defect and pulmonary arterial hypertension; 92 patients with borderline PVRI and 91 patients with low PVRI. Borderline pulmonary vascular resistance index was not associated with increased risk of postoperative mortality (p = 0.621; OR0.48, 95% CI 0.04-5.48), but associated with higher risk of overall morbidity in bivariate analysis (p = 0.002; OR3.28, 95% CI 1.5-6.72). Multivariate analysis showed positive association of borderline pulmonary vascular resistance index (p = 0.045; OR2.63, 95% CI 1.02-6.77) and preoperative tricuspid valve gradient ≥64 mmHg (p = 0.034; OR2.77, 95% CI 1.08-7.13) with overall morbidity. Conclusion: There is no difference in incidence of in-hospital mortality between preoperative borderline and low pulmonary vascular resistance index patients. However, preoperative borderline pulmonary vascular resistance index and tricuspid valve gradient ≥64 mmHg are associated with increased overall morbidity after surgical closure in secundum atrial septal defect patients with pulmonary arterial hypertension.

Pena-Shokeir syndrome's first case report from Syria.

Pena-Shokeir syndrome is considered to be a fatal congenital condition that is rarely diagnosed in neonates. We present the first-ever reported case of Pena-Shokeir syndrome from Syria. Clinical assessment and early prenatal diagnosis are both needed to give the mother and baby more realistic options.

Late presenting partially displaced atrial septal closure device: Surgical vs percutaneous correction? A case report.

INTRODUCTION: Atrial Septal Defect (ASD) is one of the most common congenital cardiac defect. Even though surgical repair of ASD is the current method of choice but percutaneous device closure is rapidly gaining popularity as it is less invasive. Dislodgment and embolization of the device may occur requiring urgent surgical retrieval. CASE PRESENTATION: We report a case of 54-years-old female patient with a history of ASD device closure 4 years ago, presenting with progressive shortness of breath for past 2 months. She had a partial dehiscence of an ASD device causing a residual ASD of 17 mm. She underwent urgent surgical repair of an ASD with a bovine pericardial patch without ASD device being explanted. CLINICAL DISCUSSION: Management of a dislodged ASD device may be percutaneous or surgical. Dislodged ASD devices that present months after deployment may become fibro-adhered to the site of embolization. Hence its retrieval can be challenging even via open surgical method. Our case describes a novel method to repair a residual ASD and prevent complications associated with dislodgement of device without completely explanting the device. CONCLUSION: In this case, the late presentation of the patient with a partially dehisced device makes it a distinctive case with a novel way on how to treat such a presentation surgically, ensuring that the device doesn't embolize further causing fatal complications.