History Of Atrial Septal Defect Research Articles

Abstract 17755: Post-Cardiac Injury Syndrome From Robotic Atrial Septal Defect Closure: A Case of Recurrent Pericarditis

Case Presentation: A 23-year-old female rugby player presented for recurrent chest pain. She has a history of atrial septal defect (ASD) repaired with robotic ASD closure. She developed acute pericarditis postoperatively and was treated with naproxen and colchicine inpatient. Her subsequent recurrences occurred with playing rugby and were treated with ibuprofen, colchicine, and prednisone. Electrocardiogram showed sinus bradycardia at 59 beats per minute but without ST segment changes. Cardiac magnetic resonance (CMR) imaging showed mild-to-moderate circumferential pericardial late gadolinium enhancement (LGE) without signal hyperintensity on T2 STIR imaging. She was diagnosed with chronic recurrent pericarditis secondary to post-cardiac injury syndrome (PCIS) and was started on rilonacept. On follow-up after 1 year, the patient remained symptom-free. She gradually increased her activity level to walking 3 miles per day and weaned down to rilonacept monotherapy. Repeat CMR showed mild pericardial LGE that was improved from before. Discussion: PCIS is a known cause of pericarditis due to local and systemic inflammatory responses to cardiothoracic surgery. Robotic ASD closure is a minimally invasive endoscopic procedure that has a favorable safety profile; PCIS is not a previously documented complication. Treatment of pericarditis secondary to PCIS includes NSAIDs plus 3 months of colchicine for acute pericarditis, NSAIDs plus 6 months of colchicine for a first recurrence, with corticosteroids or interleukin-1 inhibitors added for further recurrences. Incomplete treatment of acute pericarditis, as in this patient's case, is a risk factor for recurrent pericarditis. The risk of recurrences with physical activity is debated and current guidelines recommend restriction from physical activity until there is no evidence of active disease, though the data is not robust. In clinical practice, a gradual return-to-play program is frequently utilized.

Acute electrocardiographic correlates post heart transplant in a non-compacted myocardium obese patient: A case report

This work describes a patient with non-compacted myocardium with a very low ejection fraction, type I obesity, with a history of atrial septal defect (ASD) correction, who underwent heart transplantation and the immediate electrocardiographic changes after the procedure. The immediate alterations included atrial fibrillation with a high ventricular response, bradycardia requiring pacing, and alterations due to right ventricular pressure overload (right bundle branch block). After the initial perioperative course, we observed good evolution after transplantation and clinical treatment. At the one-year follow-up, the patient remained without signs of rejection and with grade I functional capacity, free of cardiovascular events.

P269 AORTA–RIGHT ATRIAL TUNNEL POST TRICUSPID ANNULOPLASTY

Abstract Background Aorta–right atrial tunnel is a rare entity characterized by a iatrogenic vascular fistula, which connects aortic root and right atrium. It is frequently associated with congestive heart failure (HF). Case A 54 y female with an history of atrial septal defect (ASD) type ostium secundum closure and a recent mitral and tricuspid annuloplasty surgery, presents to the hospital with significant decrease in exercise tolerance and progressive shortness of breath. At the presentation, physical examination was significant for tachycardia, continuous murmur over precordium and lower extremity edema. ECG showed right ventricular pressure overload with repolarization abnormality. TTE showed moderate systolic flattening of the septum and dilated right sections. Non coronary cusp of aortic valve demonstrated a perforation extending to the right atrium (RA). ETE confirmed the connection between non–coronary aortic cusp and RA, furthermore revealed unkown sinus venosus type ASD. TC angiography showed a marked dilation of the pulmonary artery trunk (egg and banana sign) and a connection between aorta and RA. She was treated with surgical closure of aorta– right atrial tunnel and ASD through detached and pledget stitch closure. Follow–up reveals significant functional recovery of shortness of breath and exercise tolerance. Discussion Aorta– right atrial tunnel is a rare entity characterized by a vascular fistula, which connects one of the sinuses of Valsalva and the RA. It is a rare complication of tricuspid plastic surgery. It is classified according to its relation to the aorta to anterior or posterior. The clinical presentation ranges from asymptomatic continuous murmur to the symptoms of right sided HF. In our case, the presence of ASD accelereted and worsened the manifestations of right HF. It is usually diagnosed by TTE or ETE, TC angiography and aortography. The treatment of this condition is important even in asymptomatic cases due to the risk of aortic valve dysfunction, likely regurgitation, congestive right sided HF followed by left sided HF, calcification, infective endocarditis, aneurysmal dilatation and pulmonary hypertension. Conclusion The possibility of complications of aorta–right atrium fistula necessitates early therapy, even in asymptomatic patients or those with a hemodynamically insignificant shunt. Available treatment is surgical closure. It has a high mortality rate if it is not diagnosed adequately and surgically treated without delay.

Aniridi ile İlişkili Konjenital Glokomlu Bir Olguda Alt Kapak Epiblefaron Cerrahisi: Olgu Sunumu

Aniridi, kromozom 11p13 üzerinde bulunan PAX6 genindeki bozukluğa bağlı gelişen kısmı veya tam iris defektidir. Aniridiye konjenital katarakt, konjenital glokom, keratopati veya foveal aplazi gibi oküler anomaliler eşlik edebilir. Epiblefaronun etyopatojenezi tam olarak bilinmemektedir. Bununla beraber epiblefaron etyopatogenezinde konjenital cilt katlanma anomalisi ve alt kapak retraktörlerinin yanlış yapılanması olduğu düşünülmektedir. Karotiko-kavernöz fistül, tiroid oftalmopati, megalo-oftalmi ve konjenital glokom gibi oftalmolojik hastalıklarla birlikteliği bildirilmiştir. Bu çalışmada aniridiye bağlı konjenital glokom ile takipli Doğu-Asyalı dört yaş erkek olguda gelişen epiblefaronun tanı ve tedavisinin sunulması amaçlandı. Özgeçmişinde atrial septal defekt bulunan hastanın her iki gözünde alt kapakta epiblefaron, aniridi, mikrosferofaki ve katarakt mevcuttu. Bu olguya epiblefaron nedeniyle alt kapak retraktörleri ile orbiküler kası hedef alan cerrahi teknik uygulandı.

Brain abscess due to Aggregatibacter aphrophilus in association with atrial septal defect：Case report and literature review

BackgroundAggregatibacter aphrophilus（A. aphrophilus）is one of the organisms of the HACEK group. Previously reported cases of brain abscesses caused by A. aphrophilus infection have occurred in children with a basis for congenital heart disease, or in adults with a basis for dental disease. Rare cases of brain abscess caused by A. aphrophilus have been reported in adults with congenital heart disease or in patients without dental disease history. Herein we present a rare case of brain abscess caused by A. aphrophilus, who was in association with atrial septal defect for more than 20 years, and had no dental disease and did not develop infective endocarditis. Case presentationA 51-year-old female was admitted due to progressively worsening headache and left limb weakness for more than 10 days. She denied the history of chronic diseases such as hypertension and diabetes, and no periodontal disease. While she had a history of atrial septal defect, a form of congenital heart disease with severe pulmonary hypertension for more than 20 years. After admission, echocardiographic illustrated congenital heart disease with severe pulmonary hypertension. CT and MRI showed brain abscess. Cerebrospinal fluid (CSF) results also confirmed the presence of intracranial infection. Empirical therapy with vancomycin 1.0 g i.v q12h and meropenem 2.0 g i.v q8h was initiated from the day of admission. On the fourth day after admission, brain abscess resection and decompressive craniectomy were performed, and the pus drained on operation were cultured and Gram-negative bacilli grew, which was identified as A.aphrophilus. Vancomycin was discontinued and meropenem was continued（2.0 g i.v q8h）for 5 weeks, followed by oral levofloxacin 0.5 qd for 4 weeks of out-patient antibiotics. The patient recovered fully within 9 weeks of treatment. ConclusionsThis is the first case of A. aphrophilus to cause brain abscess in adult with a history of congenital heart disease for more than 20 years, who had no dental disease and did not develop infective endocarditis. We also highlight the value of bacterial 16 S rDNA PCR amplification and sequencing in identifying bacteria in abscesses which are culture-negative, and prompt surgical treatment，choosing effective antibiotics and appropriate course of treatment will get better clinical effect.

Combined branch retinal vein and branch retinal artery occlusion in atrial septal defect

<br>A 30-year-old male patient presented with branch retinal artery occlusion and branch retinal vein occlusion in his right eye. The patient had a history of atrial septal defect (ASD). A cardiology consultation was sought, and the patient was admitted for closure of moderate-grade ASD. Subsequently, his retinal hemorrhage showed improvement. Patients with branch retinal artery occlusion and branch retinal vein occlusion affecting the same retinal quadrant should be examined for heart issues to rule out an atrial septal defect.<br>

Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKH) Patients Treated with Laparoscopic Neovaginal with The Davydov Procedure with Epidural Anesthesia: A Case Report

Introduction. MRKH syndrome is characterized by incomplete or absent of vaginal and uterine agenesis. Currently there are no official guidelines on the management of MRKH syndrome. This case report is about the operative management in MRKH patients by neovaginal with laparoscopic Davydov procedure.&#x0D; Case presentation. A 26-year-old woman admitted that she had never had a period, the patient admitted that her breasts began to grow at the age of 13, and the patient was planning to get married, and refused to take further planning. The patient then came back to the RSMH urogynecology clinic after marriage, from the examination it was found that the pubic hole was ± 0.5 cm deep, speculum examination could not be done, the uterus could not be palpable from the rectal toucher. From the ultrasound examination, it was found that both ovaries were within normal limits and suspected uterine hypoplasia. Hormone tests were within normal limits, with the following values: LH 27.01 mIU / mL, FSH 57.08 mIU / mL, Estradiol 10 pg / mL, Prolactin 5.94 ng / mL. Patients with a history of atrial septal defect (ASD) and ventricular septal defect (VSD)&#x0D; Conclusion. Treatment of MRKH syndrome patients is usually complex and requires multidisciplinary counseling, therapy, and management, so it is hoped that it can help patients to make a functional vagina. Further research and collaboration from various multidisciplinary make pregnancy in patients with MRKH syndrome, not impossible, along with current technological and scientific developments, the selection of surrogate mothers, and uterine transplantation is an option.

Cardiac Tamponade Caused by Cutibacterium acnes: An Updated and Comprehensive Review of the Literature.

Bacterial pericarditis is a critical diagnosis caused by a wide range of organisms including Streptococcus pneumoniae and other anaerobic organisms like Cutibacterium acnes which has been gaining more importance as a causative organism. Cutibacterium species are Gram-positive microaerophilic rods that constitute part of the normal flora of skin and mucosal membranes. The incidence of pericarditis caused by this organism is underreported as it is often dismissed as a skin flora contaminant. However, if left untreated, Cutibacterium acnes can cause pericarditis with serious complications. In this paper, we present a comprehensive review of the literature regarding pericarditis caused by Cutibacterium acnes along with a case presentation from our institution. In our institution, a 20-year-old man with history of atrial septal defect presented with chest pain radiating to the back along with symptoms of upper respiratory tract infection including headaches and myalgia. Electrocardiogram was remarkable for diffuse low-voltage waves. Echocardiography revealed a large pericardial effusion with tamponade features. Pericardiocentesis drained 1.2 L of milky fluid. Pericardial fluid analysis grew Cutibacterium acnes after being cultured for 8 days. The patient received 3 weeks of IV penicillin followed by 3 weeks of oral amoxicillin along with nonsteroidal anti-inflammatory agents and colchicine with no recurrence. Pericarditis caused by Cutibacterium acnes requires a high clinical suspicion since isolation of this organism can be dismissed as a skin flora contaminant. Literature review reveals that this infection may be underdiagnosed and underreported. Prompt diagnosis may lead to timely initiation of antibiotics which can help prevent devastating complications like constrictive pericarditis. Prospective studies are needed to evaluate the true incidence and prevalence of this disease.

Pathophysiology and natural history of atrial septal defect.

Atrial septal defects are among the third most common types of congenital heart disease. This group of malformations includes several types of atrial communications allowing shunting of blood between the systemic and the pulmonary circulations. The nature of shunting across the defect in patients with atrial septal defect is of particular interest. Understanding the underlying mechanisms of interatrial shunts and their natural history will help selecting the best timing for closure, before irreversible cardiac and pulmonary injury occur. This review describes the different pathophysiologic mechanisms involved in the direction and magnitude of blood flow through atrial septal defects. The natural history of an individual born with an isolated atrial septal defect is then discussed, including the impact of a longstanding shunt on survival.

Atrial Septal Defect Device Closure in the Pediatric Population: A Current Review

Atrial septal defects are the most common congenital cardiac defects. The natural history of an uncorrected atrial septal defect causes a shortened life expectancy due to right ventricular volume overload and associated congestive heart failure, atrial arrhythmias, and/or pulmonary vascular disease. Surgical closure of the atrial septal defect is a procedure with a long-standing history, and the maturing field of percutaneous closure of atrial septal defects by device implantation has established itself to be a feasible, minimally invasive, and safe procedure. Inherent limitations in device designs have resulted in rare, but serious complications, through subsequent changes in the technical aspects of transcatheter atrial septal defect closure that have minimized the number of patients with an adverse event. Recent Food and Drug Administration re-evaluation of the safety of atrial septal defect closure by device has brought to light some of the acute and long-term issues related to device occlusion. This paper summarizes the history of atrial septal defects, surgical and transcatheter device closure, and the most current outcomes of percutaneous atrial septal defect device occlusion in the pediatric population.

Predictors of sick sinus syndrome in patients after successful radiofrequency catheter ablation of atrial flutter.

PurposeThe identification of sick sinus syndrome (SSS) in patients with atrial flutter (AFL) is difficult before the termination of AFL. This study investigated the patient characteristics used in predicting a high risk of SSS after AFL ablation.Materials and MethodsOut of 339 consecutive patients who had undergone radiofrequency ablation for AFL from 1991 to 2012, 27 (8%) had SSS (SSS group). We compared the clinical characteristics of patients with and without SSS (n=312, no-SSS group).ResultsThe SSS group was more likely to have a lower body mass index (SSS: 22.5±3.2; no-SSS: 24.0±3.0 kg/m2; p=0.02), a history of atrial septal defects (ASD; SSS: 19%; no-SSS: 6%; p=0.01), a history of coronary artery bypass graft surgery (CABG; SSS: 11%; no-SSS: 2%; p=0.002), and a longer flutter cycle length (CL; SSS: 262.3±39.2; no-SSS: 243.0±40; p=0.02) than the no-SSS group. In multivariate analysis, a history of ASD [odds ratio (OR) 3.7, 95% confidence interval (CI) 1.2-11.4, p=0.02] and CABG (7.1, 95% CI 1.5-32.8, p=0.01) as well as longer flutter CL (1.1, 95% CI 1.0-1.2, p=0.04) were independent risk factors for SSS.ConclusionA history of ASD and CABG as well as longer flutter CL increased the risk of SSS after AFL ablation. While half of the patients with SSS after AFL ablation experienced transient SSS, heart failure was associated with irreversible SSS.

Atrial septal defect (secundum) with tricuspid regurgitation in a young adult cadaver

This report describes a case of a young adult cadaver found to be having atrial septal defect (ASD) of secundum type along with tricuspid regurgitation during routine dissection by medical students. The ASD had a maximum diameter of 8 mm. The report discusses the embryological development of the interatrial septum along with the possible development of this anomaly. The article goes on to discuss the natural history of ASDs of the secundum type, the modes of investigation used today and the significance of socioeconomic status in relation to ASD.

Atrial Septal Defects in the Elderly: A Study of Clinical and Echocardiographic Predictors of Atrial Septal Defect Closure

A paucity of data exists on the natural history of atrial septal defects (ASD) in the elderly. We reviewed our echocardiography database to evaluate the clinical and echocardiographic variables that might portend ASD closure.

Effects of Transcatheter Closure of Atrial Septal Defects on Heart Rate Variability

This study evaluated heart rate variability and its changes in 30 patients before and after transcatheter closure of secundum atrial septal defects. Heart rate variability data from 30 healthy volunteers with normal echocardiographic parameters and no history of atrial septal defects were included as controls. Values for the SD of all the normal RR intervals (SDNN), the SD of the means of all the 5-min segment normal RR intervals (SDANN), and the mean of all the 5-min SDs of normal RR intervals during the 24-h period (SDNN index) in patients with atrial septal defects before transcatheter closure were statistically significantly different from controls. At 6 months after closure of the defects these values were not statistically different from controls. It is concluded that transcatheter closure of secundum atrial septal defects had positive effects on heart rate variability and, consequently, may contribute to less mortality and morbidity.

Atrial septal defect: waiting for symptoms remains an unsolved medical anachronism

This editorial refers to ‘Benefit of atrial septal defect closure in adults: impact of age’[†][1], by M. Humenberger et al ., on page 553 During the past decade a ‘more modern’ natural history of atrial septal defect (ASD) in adults has been written, but still the ASD remains by far the most underdiagnosed congenital heart disease in the adult age group. The moment of diagnosis within the adult population is mostly tantamount to the initiation of treatment. Due to the superiority of closure of the defect over medical treatment alone,1 the consequence is closure either by intervention in selected patients or by surgery for all unselected or at least for the remaining patients not treatable by intervention. Meanwhile supported by direct comparison of the two methods, interventional treatment in selected patients has been accepted as the standard treatment and the treatment of choice due to its lesser invasiveness and lower complication rate.2,3 The study by Humenberger et al. 4 shows nicely in a relatively large cohort of adult patients with an age range of 20–80 years that the defects were able to be closed successfully by intervention regardless of age, with excellent results and no significant complications. New York Heart Association functional class improved in almost all patients and rapid right ventricular remodelling occurred with a statistically significant improvement in right ventricular measurements and a decrease in pulmonary artery (PA) pressure levels in those with formerly elevated pressures and resistances. The authors again affirm the findings of numerous publications during the past decade and also uniformly … [1]: #fn-2

DDAVP Versus vWF-Containing Factor Concentrate in a Patient with ET Associated Acquired von Willebrand Syndrome (AVWS).

The patient is a 16 year old boy with a past medical history of atrial septal defect and juvenile migraine headaches. He had no unusual bleeding or bruising during his early childhood including circumcision. His family history was negative for bleeding diasthesis. At the age of 12, he was diagnosed with an intramuscular hematoma on MRI after a baseball injury. Four months later he had heavy bleeding after an accidental knife wound to his hand, requiring repeated suturing. At age 14, he had another intramuscular hematoma after baseball injury, impairing range of motion in his leg. At age 15, following another deep muscular hematoma, he underwent a laboratory evaluation for the first time. His CBC was normal except for a platelet count of 1,216,000/μl and he was subsequently diagnosed with ET. He had an elevated PTT, with a normal PT. He had a FVIII level of 42%, vWF Ag of 43%, and vWF:RCoF < 20%. Multimer analysis showed relatively decreased to absent large multimers. To evaluate optimal treatment, a Stimate (DDAVP) challenge, as well as a vWF-containing concentrate (HumateP) infusion study were performed: [Display omitted] [Display omitted] The patient demonstrated a good response to DDAVP, with greater than 2-fold baseline levels of FVIII and VWF:RCo persisting after 4 hours. Multimer studies showed increases of low and intermediate multimers after administration of DDAVP. Following infusion of Humate P (53 vWF:RCo U/kg), VWF:Ag levels increased appropriately; however, VWF:RCo and FVIII levels increased to a lesser extent and fell to baseline by 4 hours. Platelet counts remained stable with both Stimate and Humate P. These results suggest that the patient's platelets have an especially high affinity for high molecular weight (HMW) multimers, explaining the lack of HMW multimers on baseline analysis in this patient. The shortened response duration following Humate P infusion suggests rapid clearance due to platelet binding of the larger (and more functional) multimers contained in the concentrate. The longer duration of response to DDAVP, which releases his own low and intermediate multimers (but not HMW multimers) into circulation, indicates that DDAVP may be a superior treatment option in patients with AVWS associated with ET.

Flattening of the interventricular septum (D-shaped left ventricle) in addition to high right ventricular tracer uptake and increased right ventricular volume found on gated SPECT studies strongly correlates with right ventricular overload

Flattening of the interventricular septum (D-shaped left ventricle) detected during echocardiographic examination is correlated with significant right ventricular (RV) overload. There are no reports of this finding with cardiac gated single photon emission computed tomography (SPECT) imaging. We report an observational study correlating this finding with the presence of RV overload. Retrospectively, we compared 8 cases with flattening of the interventricular septum on cardiac gated SPECT imaging for which echocardiographic correlations and clinical data were available regarding the presence of RV overload. All patients but 1 had pulmonary hypertension ranging from 42 to 52 mm Hg measured by echocardiographic Doppler studies. All patients but 1 had reasons for RV overload (chronic obstructive pulmonary disease in 3, history of atrial septal defect in 3, pulmonary embolism in 1, and obstructive sleep apnea in 1). Septal flattening present on gated SPECT images was seen in 50% of the cases by echocardiography. Other signs of RV overload (RV enlargement, RV hypertrophy) were observed by echocardiography in 5 patients and by the gated SPECT in 7 patients. The presence of interventricular septal flattening on gated SPECT studies correlates with RV overload and should be routinely assessed during interpretation of gated SPECT studies.

LEFT-TO-RIGHT SHUNT LESIONS

A left-to-right shunt lesions exists when blood from the left atrium, left ventricle, or aorta transits to the right atrium or its tributaries, the right ventricle, or the pulmonary artery. This article discusses: the incidence, types, embryology, clinical presentations, physical examinations, electrocardiographic features, chest radiographs, echocardiographic and cardiac catheterization issues, treatment, natural history of atrial septal defects, and outcomes of treatment of atrial septal defects, ventricular septal defects, patent ductus arteriosus, and endocardial cushion defects.

Natural history of atrial septal defect in young infants

Natural history of atrial septal defect in young infants

Effects of corrective surgery on natural history of atrial septal defect of secundum type.

Two hundred and seventy-eight patients with atrial septal defect of secundum type were operated on surgical closure of the defect. The patients were divided into 5 groups according to age at surgery, and preoperative complaints and laboratory findings were analyzed to evaluate natural history. As year passed, incidence of complaints increased. Cardiothoracic ratio (CTR), systolic pressure of the right ventricle (PRVS), end-diastolic pressure of the right ventricle (RVEDP), mean pressure of the right atrium (PRAm), ratio of pulmonary to systemic systolic pressure (PPA/PS), right ventricular dimension (RVD) and left atrial dimension (LAD) increased significantly, while frontal axis of the QRS complex, RV1, SV1 +RV5 and LVD decreased. No chronological changes were seen in ratio of pulmonary to systemic blood flow (QPA/QS) and ratio of pulmonary to systemic vascular resistance (RPA/RS). The first and second decades of life were considered to be stable stages of the disease. Postoperatively, changes in the above-mentioned parameters were compared in each age group, and effects of surgical repair on natural history were evaluated. Of 265 survivors, 168 were followed-up for more than one year, the longest period being 22 years. CTR, frontal axis, RV1, PRVS, RVEDP, PPA/PS and RVD decreased significantly, while SV1 + RV5, RPA/RS and LVD increased. No changes were seen in LAD. Surgical effects appeared most significantly in the first decade and least in the fifth or more. From these findings it would be concluded that corrective surgery should be best carried out in the first decade, at latest in the second, though not to be contraindicated by age alone. Otherwise, postoperative improvement of parameters delays or hemodynamical abnormalities may persist regardless of considerable clinical improvement.