History Of Congenital Heart Disease Research Articles

Aortic Pulse Wave Velocity Derivation From Arterial Waveforms in Critically Ill Infants and Children

Methods Hemodynamically stable pre-pubertal children with an indwelling arterial catheter were enrolled. Applanation tonometry was used to measure aPWV, which was derived from the transit distance and pulse transit time. The carotid and femoral arteries were used as preferential sites and transit distance was measured relative to the suprasternal notch, an anatomical landmark for the aortic valve. Pulse transit time was derived from the time delay between the two pulse waveforms from the ECG-R wave. Peripheral arterial line waveforms were converted into ascending aortic waveforms by Hemolab software. Forward and backward wave forms were derived from the ascending aortic waveforms. Time delays were calculated between forward and backward wave forms. Effective reflecting distance (EfRD) was calculated from the equation 0.173*age+0.661*BMI+ 34.548. Estimated aPWV was derived from EfRD and the time delays. Measured aPWV and estimated aPWVs were compared. Results Aortic pulse wave velocity and arterial pressure waveforms were obtained in 33 infants and children (50% female). 66% (22) patients had history of congenital heart disease. 54% (18) patients were on mechanical ventilation. 39% (13) patients were on vasoactive medications. 0-1 year aged infants (n=18) had aPWV 0.49-15.91 m/s. aPWV in 1-5-year-old patients (n=5) was 1.82-3.72 m/s, and in 6-10-year-old patients (n=6) was 1.27-4.09 m/s. 10-12-year-old patients (n=4) had aPWV 1.7-7.02 m/s. We identified artifacts from ventilators, nasogastric lines, and pacifiers in some patients that necessitated the use of the brachial artery instead of the carotid artery for the measurement of aPWV. Measured and estimated (from arterial waveforms) aPWVs were similar in 6 patients. Estimated aPWV from arterial waveforms by using Effective reflecting distance (EfRD) correlated well with the patients' age, weight, and length. Conclusions Aortic pulse wave velocity can be feasibly measured in a critical care setting, although patient monitoring and instrumentation may make uniform measurement protocols not feasible. Future derivations of aPWV from arterial waveforms will need to take this into account. Calculating aPWV from arterial waveforms can help us to include a larger sample size of the patient population with arterial waveform data, eliminating the need for tonometry.

Congenital heart defects and skeletal malformation syndrome with congenital hemiplegia

Congenital heart defects and skeletal malformation syndrome is very rare syndrome. Most of the patients had germline mutations in ABL1 gene. A 30-year-old gentleman presented with history of congenital heart disease (ventricular septal defect) and skeletal malformations which are typical of CHDSKM. Patient also had congenital hemiplegia which is rare in CHDSKMS. Patient also had lactose intolerance since childhood. Patients were evaluated thoroughly to rule out other causes. Current report is one of the rare case reports of CHDSKMS, only few case reports have been published till now.

Coronary Allograft Vasculopathy is Associated with Decreased CD34+ Cell Count in Heart Transplant Recipients

Purpose The underlying mechanisms of coronary allograft vasculopathy (CAV) after heart transplantation remain incompletely understood. Since CD34+ cells represent one of the key determinants of coronary vascular homeostasis we investigated the potential association between CAV and CD34+ cell count in heart transplant recipients. Methods In a single-center prospective pilot study we included 18 adult heart transplant recipients without history of congenital heart disease, multi-organ transplantation or oncologic therapy. All patients underwent coronary CT angiography and CAV was defined in accordance with the ISHT criteria. At the time of CT angiography we collected blood samples and measured CD34+ cell count using Beckman-Coulter Navios EX flow cytometry according to ISAGE protocol. Results CAV was present in 6 patients (33%; Group A) and absent in 12 patients (67%; Group B). The two groups did not differ in age (58±8 years in Group A vs. 53±14 years in Group B,P=0.46), gender (male: 100% vs. 83% in Group B,P=0.31), underlying disease etiology (ischemic: 67% vs. 33%,P=0.20), presence of hypertension (83% vs. 75%,P=0.70), diabetes (50% vs. 33%,P=0.52) or renal insufficiency (17% vs. 33%,P=0.48). Furthermore, donor age (45±9 years in Group A vs. 38±10 years in Group B,P=0.22), allograft ischemic time (205±68 min vs. 193±63 min,P=0.72), tacrolimus trough levels (6.9±1.3 μg/L vs. 6.8±1.2 μg/L,P=0.76), and NT-proBNP levels (478±288 pg/mL vs. 473±373 pg/mL,P=0.97) were comparable. Though total leukocyte count was similar (7.5±3.4 × 109/L in Group A vs. 6.8±2.1 × 109/L in Group B,P=0.60), we found significantly lower CD34+ cell count in Group A compared to Group B (1.37±0.36 × 106/L vs. 2.15±0.81 × 106/L,P=0.04). When stratifying patients according to CD34+ cell count, patients with cell count below the median displayed increased incidence of CAV compared to patients with cell count above the median (55% vs. 11%,P=0.04). Overall, patients with CAV ISHLT score>1 had lower CD34+ count when compared patients with ISHLT grade 1 CAV (0.91±0.06 × 106/L vs. 1.32±0.14 × 106/L,P=0.03). Conclusion Lower CD34+ cell count appears to be associated with CAV in heart transplant recipients. Further clinical trials are warranted to better define the underlying mechanisms and investigate the potential of CD34+ cells in the prevention and treatment of CAV in this patient cohort.

The Predisposing Risk Factors for Non-syndromic Congenital Heart Disease: A Case-Control Study

Objectives: Despite the importance of congenital heart disease (CHD) in the population, the risk factors predisposing infants to be born with non-syndromic CHD are not well understood yet. Materials and Methods: In a case-control study, we recruited consecutive 109 infants with non-syndromic CHD who were referred to our pediatric cardiac care clinic and then compared them with 117 infants without CHD (2015-2016). Paternal, maternal, and neonatal demographic information, maternal past medical history, and antenatal history were recorded for each child. To investigate the potential risk factors for developing CHD, infants with and without CHD were compared in terms of study variables. In a second analysis, preterm infants were excluded and term infants were compared regarding the study variables. Results: The findings revealed that higher maternal and paternal education were both associated with a lower risk of CHD (odds ratio [OR]: 0.47, 95% confidence interval [CI]: 0.24-0.93, P=0.031, and OR: 0.45, 95% CI: 0.23-0.89, P=0.023, respectively). The family history of CHD in the first- or second-degree relatives of infants was significantly associated with CHD (OR: 3.56, 95% CI: 1.35-9.40, P=0.007). Several parameters were more prevalent in the CHD group, including having lower birth weight, having preterm birth, being the fourth birth order or higher, and not receiving maternity care under the supervision of a gynecologist. However, higher birth order and lower birth weight were not associated with CHD in exclusively term infants. Finally, a higher maternal educational level was related to lower CHD in term infants even after adjusting for a family history of CHD and preconception diabetes mellitus (OR: 5.45, 95% CI: 1.71-17.37, P=0.004). Conclusions: Our study findings demonstrate the need for a more enhanced primary care program, especially in patients with poor financial status and a family history of CHD.

Use of Fenoldopam in Children with Congenital Heart Disease to Decrease Fluid Balance: A Retrospective, Descriptive Study and Insights into Predictors of Decreased Fluid Balance.

This retrospective study aimed to determine if fenoldopam is associated with a decrease in fluid balance and to define the factors that may promote this in children with a history of congenital heart disease at the cardiac intensive care unit (CICU). Patients cared from January 2014 to December 2018 in the CICU were reviewed, and those on fenoldopam infusion were identified. Patient cohort data included demographics, clinical information, laboratory results, hemodynamic and urine output measurements, and information regarding fenoldopam infusion were compared between those with and without decrease in fluid balance. Forty-six patients were identified. Patients received a starting dose of fenoldopam of 0.2 mcg/kg/h, a maximum dose of 0.3 mcg/kg/h, and duration of 64 hours. Over the 4-hour study period, statistically significant change was noted in systolic pressure (decrease of 5.4%; p < 0.001), diastolic pressure (decrease of 3.5%; p = 0.01), fluid balance, and urine output (decrease of 1.3%; p = 0.027). In the cohort, 34 patients (74%) had a decrease in fluid balance, 18 (39%) had an increase in urine output, and 25 (54%) had a decrease in fluid input after the initiation of fenoldopam. Patients that had a decrease in fluid balance tended to have a higher blood urea nitrogen level at the time of fenoldopam initiation. Fenoldopam was associated with decrease in fluid balance and fluid input, but not associated with an increase in urine output. The identification of factors that can decrease fluid balance may help identify those patients who can be benefited with this treatment.

A Teenage Boy with Right Forehead Swelling Following Trauma to the Head.

1. Aviva P. Aiden, MD, PhD*,†,‡ 2. McKenna E. Boyd, MS§ 3. John M. Carey, MD* 4. Shabana Yusuf, MD, MEd* 1. *Division of Pediatrics Emergency Medicine, Department of Pediatrics, 2. †Department of Molecular & Human Genetics, Baylor College of Medicine and Texas Children’s Hospital, Houston, TX; 3. ‡Department of Bioengineering, Rice University, Houston, TX; 4. §Office of Student Affairs, Baylor College of Medicine, Houston, TX 1. Address correspondence to Shabana Yusuf, MD, MEd, Associate Professor of Pediatrics, Division of Pediatrics Emergency Medicine, Department of Pediatrics, Baylor College of Medicine and Texas Children’s Hospital, 6621 Fannin St A2210, Houston, TX 77030-2399. E-mail: syusuf{at}bcm.edu A 15-year-old boy presented to the emergency department (ED) with headache and swelling to his right forehead after a fall. The fall was unwitnessed, but the patient reports he hit his head and briefly lost consciousness. It was unclear if the patient had a syncopal event leading to the fall, or had a loss of consciousness due to the head injury resulting from the fall. On review of systems, he reported a headache that had started after the fall earlier that day, and nasal congestion with rhinorrhea for the past several days. Almost 2 weeks before, he had a headache which lasted for 1 day. The remainder of the review of systems was negative. The patient reported a vague history of a cardiac problem, and the patient’s mother reported that he was supposed to avoid sports, but had not been evaluated by a cardiologist and had not been prescribed any medications. No records were available regarding this medical history, and the patient and parent were unable to elaborate further. There was no family history of congenital heart disease, sudden death, or arrhythmias. On arrival to the ED, he was febrile to 102°F (38.9°C) and tachycardic at 118 beats/min. On physical examination, he was noted to have tender right frontal scalp swelling without fluctuance. He had nasal congestion but no other facial tenderness and normal dentition. His mental status, neurological examination, and the remainder of his physical examination including his cardiac and lung examination were all normal. Evaluation with noncontrast computed tomography (CT) scan of the head revealed “paranasal sinus disease with fluid levels in the frontal …

Factors Associated with Congenital Heart Diseases among Children in Uganda: A Case-Control Study at Mulago National Referral Hospital (Uganda Heart Institute)

Congenital Heart Diseases (CHD) are among the leading causes of morbidity and mortality associated with congenital malformations among children. Not knowing the risk profile of CHD among children in Uganda impedes development of effective prevention interventions. In this hospital based unmatched case-control study we examined risk factors for all types of CHD among 179 pair of case and control children aged 0-10 years old at Mulago National Referral Hospital. Odds ratios and their corresponding 95% confidence intervals were calculated using multivariate logistic regression. Low birth weight (adjusted OR: 3.15, 95% CI 1.48 - 6.69), high birth order ≥5^th birth order (adjusted OR: 3.69 (1.10 – 12.54), maternal febrile illness during pregnancy, maternal and paternal alcohol consumption, and paternal socio-economic status were associated with CHD. Family history of CHD, maternal education level, maternal chronic illness, and paternal education level were not associated with CHD. The results suggest: low birth weight, high birth order, and maternal febrile illness during pregnancy, parental alcohol use and paternal socio-economic status as dominant risk factors for CHD among children. Rigorous implementation of public health policies and strategies targeting prevention of febrile illness during pregnancy, maternal malnutrition, parental alcohol consumption, delivery of high number of children per woman, might be important in reducing the burden of CHD among children in Uganda.

Additive value of positron emission tomography/computed tomography scan for detection of aortic tube graft infection: a case report.

BackgroundDiagnosis of aortic graft infection is challenging, and delayed diagnosis is associated with poor prognosis. Positron emission tomography/computed tomography (PET/CT) has improved diagnostic accuracy.Case summaryA patient with a history of congenital heart disease was admitted due to fever. He had a history of four cardiac surgeries, including the Bentall procedure for endocarditis. Blood cultures were negative. A semi-mobile mass was detected in the distal portion of the aortic tube graft in echocardiography. PET/CT scan was used to confirm tube graft infection and to support proceeding to cardiac surgery.DiscussionUsing multimodality imaging, including PET/CT scan in combination with echocardiography, can improve diagnostic accuracy for the detection of aortic tube graft infection, infection of prosthetic valves, or intra-cardiac devices, especially in high-risk surgical cases.

Prevalence of Factors Associated with Congenital Heart Disease

Background: Congenital heart disease (CHD) is the most common congenital disorder in neonates. Many factors are associated with the occurrence of the CHD, including genetic factors, teratogenic exposure and several other unrecognized factors. Objectives: This study aims to investigate the role of several potential factors associated with the occurrence of CHD. Methods: This study was conducted as a cross-sectional case-control study, in Imam Khomeini Hospital Complex, Tehran, Iran. A total of 1338 known cases of CHD, diagnosed by echocardiography or angiography and 1201 healthy children as the controls were included in this study. Factors that could have an association with CHD were investigated by a questionnaire from all participants. Results: Maternal age above 30 years (P-value &lt; 0.001), positive parents’ consanguinity (P-value &lt; 0.001) and previous maternal history of abortion (P-value &lt; 0.001) was significantly higher among patients with CHD; furthermore, positive history of CHD among siblings of the known cases of CHD was higher than healthy ones (P-value &lt; 0.001). The mean birth weight in case and control group were 3 (+ 0.648) and 2.9 (+ 0.707) kilograms, respectively. The maternal age in 983 (73.4%) cases and 960 (79.93%) controls was below the 30 years, the maternal age for other participants was above the 30 years (P-value &lt; 0.001). Conclusions: All factors except the history of stillbirth, maternal underlying disease and drug use during pregnancy in the case group were significantly different with the control group. It seems that the causes of CHD in Iran are most commonly related to the hereditary genetic factors, and the increased maternal age, drug use, and underlying diseases in the mother are a matter of less importance.

Risk Factors and Patient Profile of Infective Endocarditis due to Gemella spp.

Background.The diagnosis of infective endocarditis is difficult, especially when it involves atypical organisms. Therefore, our study identified risk factors of infective endocarditis caused by rare pathogen, Gemella spp.Methods.A systematic review was conducted to investigate characteristics of endocarditis patients infected with Gemella spp. using the search term “Gemella” and “endocarditis.” Case reports were gathered by searching Medline/Pubmed, Google Scholar, CINAHL, Cochrane CENTRAL, and Web of Science databases. 83 articles were selected for review.Results.Five species of Gemella were identified. Typical patients were males between 31 and 45 years of age. On admission, patients had fever, tachycardia, and normal blood pressure. Common clinical manifestation other than fever included fatigue and weakness, chills and sweating, and nausea, vomiting, diarrhea, and weight changes. One in four reported a history of congenital heart disease, and a recent oral cavity infection. Laboratory tests reveal anemia, leukocytosis, and elevated erythrocyte sedimentation in all age groups, elevated C-reactive protein is observed among adult and geriatric populations only. Mitral and aortic valves were most commonly infected by Gemella spp.. The most common Gemella spp.-susceptible antibiotics were penicillin, vancomycin, cephalosporin, macrolide, and aminoglycosides. However, antibiotic resistance was observed against penicillin, aminoglycoside, and fluoroquinolone. Antibiotic course of at least six weeks resulted in superior clinical improvements than durations under six weeks. Finally, one in two patients underwent valve replacement or repair, with common complications affecting the cardiovascular, neurological, and renal systems. Finally, death occurred in 1 in 8 patients, half of which occurred post-surgical procedure, and the majority occurring equal to or greater than 1 week from admission.Conclusion.Our systematic review highlights the importance of considering rare pathogens, particularly in the presence of predisposing risk factors.

Abstract 16097: Impact of Device Miniaturization on Implantable Loop Recorder Utilization in the Pediatric Population: An Analysis of the Marketscan Database

Introduction: Implantable loop recorders (ILRs) are an effective technology in the detection of paroxysmal arrhythmias, but utility in the pediatric population has been limited by device size. In 2014, a miniaturized ILR was introduced with a less invasive implant technique. The impact of this technology on ILR utilization in pediatric patients has not been evaluated. Hypothesis: We hypothesize an increase in annual pediatric ILR implants since 2014 due to device miniaturization. Methods: A retrospective observational study was conducted using administrative claims from Marketscan Medicaid and Commercial insurance claims databases. Utilization of ILR between 1/2013 and 12/2018 was measured (normalized to the total enrolled population ≤18 years) and compared to two balancing measures (Holter ambulatory monitors and encounters with syncope as a diagnosis). Secondary analyses included evaluations of subsequent interventions and complications. Results: The study cohort included 33,532,185 individual subjects, of which 769 underwent ILR implantation. ILR subjects were 52% male, with a median age of 16 years (IQR 10-17 yrs). 71% had a history of syncope, 43% had a history of palpitations, and 28% had a history of congenital heart disease. Utilization of ILR increased in 2014, from 5 procedures per million enrollees in 2013 to 11 per million between 2015-2018, while balancing measures remained static (see Figure).Of 393 subjects with ≥1 year of follow-up post-implant, interventions included catheter ablation in 24 (6%), pacemaker implant in 15 (4%) and ICD implant in 7 (1.8%). Among this group, 14 (3.6%) had a device infection and 5 (1.3%) had an erosion. Conclusions: Following introduction of the miniaturized ILR, pediatric utilization has rapidly increased. The effect of this change on outcomes and value deserves further attention.

Regional Brain Growth Trajectories in Fetuses with Congenital Heart Disease.

Congenital heart disease (CHD) is associated with abnormal brain development in utero. We applied innovative fetal magnetic resonance imaging (MRI) techniques to determine whether reduced fetal cerebral substrate delivery impacts the brain globally, or in a region-specific pattern. Our novel design included two control groups, one with and the other without a family history of CHD, to explore the contribution of shared genes and/or fetal environment to brain development. From 2014 to 2018, we enrolled 179 pregnant women into 4 groups: "HLHS/TGA" fetuses with hypoplastic left heart syndrome (HLHS) or transposition of the great arteries (TGA), diagnoses with lowest fetal cerebral substrate delivery; "CHD-other," with other CHD diagnoses; "CHD-related," healthy with a CHD family history; and "optimal control," healthy without a family history. Two MRIs were obtained between 18 and 40 weeks gestation. Random effect regression models assessed group differences in brain volumes and relationships to hemodynamic variables. HLHS/TGA (n = 24), CHD-other (50), and CHD-related (34) groups each had generally smaller brain volumes than the optimal controls (71). Compared with CHD-related, the HLHS/TGA group had smaller subplate (-13.3% [standard error = 4.3%], p < 0.01) and intermediate (-13.7% [4.3%], p < 0.01) zones, with a similar trend in ventricular zone (-7.1% [1.9%], p = 0.07). These volumetric reductions were associated with lower cerebral substrate delivery. Fetuses with CHD, especially those with lowest cerebral substrate delivery, show a region-specific pattern of small brain volumes and impaired brain growth before 32 weeks gestation. The brains of fetuses with CHD were more similar to those of CHD-related than optimal controls, suggesting genetic or environmental factors also contribute. ANN NEUROL 2021;89:143-157.

Appropriate use of echocardiography for palpitations in paediatric cardiology clinics.

Identify diagnostic yield and frequency of echocardiograms for palpitation-related indications at outpatient paediatric cardiology clinics in relation to the 2014 ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE appropriate use criteria for Initial Transthoracic Echocardiography in Outpatient Paediatric Cardiology. A single-centre, retrospective study of children presenting for evaluation of a chief complaint of palpitations to outpatient paediatric cardiology clinics from 2015 to 2017. Palpitations were defined as an unpleasant sensation of rapid, irregular, and/or forceful beating of the heart. Indications for echocardiogram in patients were retrospectively classified based on the appropriate use criteria as "appropriate," "may be appropriate," or "rarely appropriate." The incidence of abnormal and incidental echocardiographic findings for each category was determined. A total of 286 patients presented with palpitations, with 128 (52% female) meeting inclusion criteria. Exclusion criteria included patients with additional cardiac complaints, prior echocardiogram, or history of congenital heart disease. Echocardiograms were performed on 36 (28%) patients. The appropriate use criteria were retrospectively applied, and indications for their performance were classified as "appropriate" (n = 4), "may be appropriate" (n = 17), or "rarely appropriate" (n = 15). Minor echocardiographic abnormalities were present in 22% (n = 8) of echocardiograms obtained for all appropriate use criteria classifications. No moderate or severe echocardiographic abnormalities were found. Incidental findings were noted in eight echocardiograms. Echocardiography in the evaluation of "rarely appropriate" and "may be appropriate" palpitation-related indications is of low diagnostic yield.

Risk factors for transposition of the great arteries in Saudi population.

Objectives:To assess potential risk factors and their effect on the development of transposition of the great arteries (TGA).Methods:A retrospective case-control study of all patients diagnosed with TGA between 1999 to 2016 at King Abdulaziz Medical City, Riyadh, Saudi Arabia. Age and gender-matched controls were selected. Risk factors, including consanguinity, gestational diabetes, family history of congenital heart disease, parental age, and maternal parity, were collected. Regression modeling was used to analyze the effects of risk factors on the development of TGA.Results:A total of 206 patients with transposition of the great arteries were enrolled in the study. Transposition of the great arteries cases were divided into simple and complex TGA. Selected healthy controls were 446. In the studied cases, consanguinity was found in 95 (46%) of cases, gestational diabetes was diagnosed in 36 (17.5%) mothers, and 35 (17%) had a confirmed family history of congenital heart disease. When risk factors of the cases were compared to the controls, consanguinity, gestational diabetes, maternal age, and parity were found to significantly increase the incidence of TGA.Conclusion:Our study revealed significant risk factors for the development of transposition of great arteries including first degree consanguineous marriages, gestational diabetes, family history of congenital cardiac anomalies, and increasing maternal age and parity. These factors increased the risk by at least 2 folds.

Open heart surgery or echocardiographic transthoracic or percutaneous closure in secundum atrial septal defect: a developing approach in one Chinese hospital

BackgroundTo study the clinical manifestations and advantages of open-heart surgery and echocardiographic transthoracic or percutaneous closure with secundum atrial septal defect (ASD). The surgeon’s learning curve was also analyzed.MethodsIn all, 115 consecutive patients with ASD from May 2013 to May 2019 were enrolled. According to the operative procedure, patients were divided into three groups: group one (open repair group) (n = 24), where patients underwent ASD repair (ASDR) under cardiopulmonary bypass (CPB); group two (closed surgical device closure group) (n = 69), where patients (six patients ≤1 y and sixteen ≤10 kg) underwent transthoracic ASD occlusion under transesophageal echocardiographic (TEE) guidance; and group three (transcatheter occlusion group) (n = 22), where patients underwent percutaneous ASD occlusion under echocardiography. The clinical features and results of each group were analyzed. All patients were telephonically followed-up after 3 months.ResultsAll the three methods treating ASD were successfully performed in our hospital. It was also a typical developing history of congenital heart disease (CHD) surgery in China. One patient in the group two was transferred to emergency surgery for occluder retrieval and CPB-ASDR. Eight patients experienced failed transthoracic or percutaneous occlusion, two of whom underwent unsuccessful percutaneous closure at another hospital. Two patients each in the groups two and three were intraoperatively converted to CPB-ASDR. Two patient in the group three was converted to transthoracic occlusion surgery. All patients were discharged without any residual shunt. The three-month follow-up also did not show any residual shunt and occluder displacement.ConclusionIn low-weight, infants, or huge ASDs with suitable rim for device occlusion, transthoracic ASD closure was successfully performed. Based on knowledge of ASD anatomy and skilled transthoracic occlusion of ASD, surgeons can perform percutaneous occlusion of ASD under echocardiographic guidance.

Cardiovascular surgery in Turner syndrome - early outcome and long-term follow-up.

Cardiovascular disease is the leading cause of death in patients with Turner syndrome (TS), and cardiovascular surgery is frequently required for management of these patients. TS is associated with medical comorbidities than can complicate the care of this patient population. To describe the cardiovascular surgical outcomes of patients with TS. A retrospective case series was compiled of 51 consecutive TS patients who had at least one cardiovascular surgery at Mayo Clinic Rochester from 1977-2017. The baseline clinical data of these patients were reviewed including demographics, medical comorbidities, congenital heart disease history, and medications. Echocardiographic reports were analyzed in detail. Operative reports and surgical hospital courses were reviewed. Long-term mortality was determined using medical records and the Social Security Death Index. Survival analysis was performed with the Kaplan Meier method. The cohort comprised 51 TS patients, average age at the time of surgery at Mayo Clinic was 28 (8-41) years, and 23 (45%) patients were under the age of 18. At the time of first Mayo Clinic surgery, 18 (35%) patients had previously undergone cardiac surgery at another institution. The most common procedures were repair of aortic coarctation in 14 (28%) patients, aortic valve replacement in 6 (12%) patients, and composite aortic root/ascending aorta replacement in 7 (14%) patients, with 7 patients undergoing repair of more than one lesion. Aortic dissection required operative intervention in 5 patients. After initial Mayo Clinic surgery, subsequent operations were required in 6 (13%) patients. Average hospital length of stay was 6 ± 2 d. There were 4 (8%) early surgical deaths. Freedom from death was 97% and 89% at 10 and 20 years, and the freedom from reoperation was 93% and 81% at 10 and 20 years. Cardiovascular surgery is associated with 8% early mortality given the medical complexity of TS patients. Those who survive to dismissal have good survival. Later cardiovascular reoperations are not rare.

P719 Complex cyanotic congenital heart disease and its complications- it is never too late to treat

Abstract Noncardiac complications significantly contribute to the morbidity and mortality of adults with congenital heart disease (CHD), mainly because life expectancy and quality of life for those born with CHD have greatly improved in the last decades. Double-outlet right ventricle (DORV) with a subaortic ventricular septal defect (VSD) associated with subpulmonary stenosis is a complex cyanotic congenital heart disease from the spectrum of tetralogy of Fallot. We present the case of a young male who was diagnosed at the age of 18 with DORV with subaortic VSD (Figure A), subpulmonary stenosis (Figure C) and mitral valve malformation (anterior leaflet cleft (Figure B) and both leaflets prolapse) with secondary moderate mitral regurgitation (MR), associated with major aortopulmonary collaterals, without pulmonary hypertension. There was no surgical correction at that moment. At the age of 26 he presented with recurrent hemoptysis and embolisation of bronchial arteries was performed. In the same year, a cerebral MRI showed signs of multiple infratentorial and supratentorial ischemic strokes. One year later, he was diagnosed with brain abscess and otomastoiditis that were surgically managed; multiple microorganisms were isolated from the two sources (M. morganii, K. pneumoniae, P . aeruginosa) and the patient received prolonged antibiotic therapy. Two months later he presented with clinical, biological and echocardiographic signs of infective endocarditis (IE) and blood cultures confirmed the diagnosis of Candida albicans associated mitral valve IE (Figure D, E, F, G). Antifungal therapy was administered for one month, with the complete disappearance of the vegetation, but worsening of the mitral regurgitation and moderate left ventricular systolic dysfunction. Thus, the patient had a clear indication for mitral valve replacement together with complete repair of the cardiac malformation that seemed still feasible as the pulmonary arteries were well developed, there was no pulmonary vascular disease and the systolic function of the RV was normal. Correction of CHD was performed in March 2019 consisting of repair of the DORV (Figure H), mitral valve replacement (33 mm bileaflet mechanical valve) (Figure I), tricuspid valve repair with RVOT remodelling. Postoperative, the patient clinical status improved significantly and transthoracic echocardiography revealed a mild residual subpulmonary stenosis (Figure J) and normally functioning mitral prosthesis. There are frequent, various complications in the natural history of congenital heart diseases, especially unrepaired. This case illustrates how vicious the circle of complications can get in a case of unrepaired cyanotic cardiac malformation and the difficulty of breaking this circle. The correction of CHD is the best prevention method and should be performed whenever it is feasible, even at an adult age. Abstract P719 Figure.

P696 The role of three dimensional echocardiography in a case of recurrent infundibular pulmonary stenosis in a surgically corrected tetralogy of fallot

Abstract Background Tetralogy of Fallot (TOF) represents approximately 7%-10% of congenital heart diseases (CHDs), and it is the most common cyanotic CHD, with 0.23-0.63 cases per 1,000 births. In our case report we are reviewing the added value of three dimensional echocardiography in the assessment of degree of infundibular and valvular stenosis in tetralogy of Fallot. Case report A 29-year-old female patient with history of congenital heart disease (Fallot’s tetrology) and history of total surgical correction at the age of 10 presented to our facility by dyspnea grade III that started 1 month before presentation .On examination; an ejection systolic murmur was heard over the pulmonary area with palpable thrill. Electrocardiogram (ECG) revealed normal sinus rhythm with a heart rate of 75bpm with right axis deviation and right ventricular hypertrophy voltage criteria. Transthoracic two dimensional echocardiography revealed increased thickness of the right ventricle (RV) free wall , and aliasing of the color Doppler flow across the right ventricular outflow tract (RVOT) with a peak systolic gradient across the RVOT 69mmHg . By performing three dimensional transesophageal echocardiography we were able to demonstrate the RVOT narrowing in RVOT enface view and by using specific software we were able to demonstrate the morphology of the pulmonary valve and the pulmonary valve orifice area and we found that the pulmonary valve is a bicuspid valve as shown and the pulmonary valve orifice area was 3.8cm2 signifying the absence of significant valvular stenosis. Conclusion 3D echocardiography in adult congenital heart disease provides unique projections from the living complex anatomy (such as en face views) and true volumetric quantification without geometric assumptions Abstract P696 Figure. Infundibular stenosis

P1302 B-cell lymphoma presenting as an ejection systolic murmur

Abstract OnBehalf Nicosia General Hospital We present the case of a young patient with large B-cell lymphoma causing severe extrinsic pulmonary stenosis. This patient presented to the emergency department with chest discomfort ,fatigue and dyspnea on minimal exertion. A loud ejection mid-systolic crescendo-decrescendo murmur with widely split S2 located in 2nd left parasternal border, raised the suspicion of pulmonary stenosis and patient underwent immediate transthoracic echocardiography as the first imaging modality of choice.On transthoracic echocardiography a large mass was seen, causing severe extrinsic pulmonary stenosis, and was further diagnosed as B-cell lymphoma after CT-guided biopsy. We discuss the high index of lymphoma suspicion in cases of pulmonary stenosis findings such as loud ejection murmur in pulmonic valve auscultation area, in otherwise healthy patients, with no history of congenital heart disease. Lymphoma and other mediastinal masses represent the most common aetiology of acquired pulmonary stenosis and should be suspected in otherwise healthy patients who present with clinical findings of pulmonary stenosis and vice-versa, pulmonary stenosis should be suspected in the presence of symptomatic mediastinal masses. Abstract P1302 Figure. Lymphoma PSAX

P648 Right heart volume overload after surgical correction of atrial septal defect sinus venosus type with partial anomalous pulmonary veins drainage

Abstract 40 year-old male with history of congenital heart disease. Atrial septal defect (ASD) sinus venosus type associated with partial anomalous pulmonary venous drainage (PAPVD) was diagnosed during childhood and surgically repaired at the age of three. Since then the patient was asymptomatic and he was lost to follow up when reached adulthood. A transthorathic echocardiogram was performed during a hospitalization because of a complicated pneumonia. A severe dilatation of right chambers and main pulmonary artery was observed. The estimated Qp:Qs by this technique was 2.0 but the atrial septum seemed to be intact. Agitated saline was administered in this procedure and no passage of microbubbles was observed. A cardiac MRI was performed because of suspicion of anomalous pulmonary venous drainage. The findings observed in the echocardiogram were confirmed and an uncommon PAPVD was demonstrated by this technique: the superior left pulmonary vein drainaged into a dilated innominated vein. Also, a small pulmonary vein from the right upper lobe emptied into a "venous conduct" (yellow circle) located posterior to the superior cava vein (SCV) that later drainaged into the right atrium under the mouth of the SVC, posterior to the interatrial septum. Surgically correctioin of the PAPVD was performed. Discussion PAPVD is a congenital heart disease characterized by the drainage of one or several pulmonary veins –but not all of them- into the right atrium or systemic veins, which leads to left-to-right shunting. The estimated incidence of this disease ranges between 0.1 and 0.2% of the general population. The right superior pulmonary vein is the most frequently involved and is commonly associated to ASD sinus venosus type. Bilateral anomalous drainage, as occurred in this case, is exceptional. Furhermore, this case highlights the importance of long-term follow-up in patients with congenital heart disease due to the fact that, even when the disease is considered to be cured, long term complications could appear compromising the prognosis of the patient. Abstract P648 Figure. Unusual PAPVD