Pattern of un-operated Grown Up Congenital Heart (GUCH) patients presenting to a Tertiary Care Cardiac Institute of Punjab.

OBJECTIVES: To identify the pattern of un-operated grown up congenital heart defects at our tertiary care cardiac institute. METHODS: This is a prospective observational study. All un-operated GUCH patients who presented to Faisalabad Institute of Cardiology (FIC) from May 2017 to 30th July 2017 were enrolled. Diagnosis was established on Transthoracic Echocardiography done by dedicated pediatric cardiologist at FIC. The mode of presentation, presenting complaints, type, severity, complications and co-morbid conditions of CHD were recorded. RESULTS: A total of 200 consecutive patients were enrolled. Mean age was 29.92 ± 11.21 years. There were 104 females (52%) and 96 males (48%). Majority of patients presented in Out-Patient Department (84%) while 16% presented in emergency (n=32). The most common cardiac anomalies were: Atrial Septal Defect (ASD) 41.5% (83), Tetralogy of Fallots (TOF) 42 (21%), Ventricular Septal Defect (VSD) 28 (14%) and Patent ductus arteriosus (PDA) 8 % (16). Cyanotic CHD was present in 43% (86) while TOF was the most common of it. The disease was of moderate complexity in 77.5% patients. Certain complications like Pulmonary hypertension 69(34.5%), Eisenmenger 33(16.5%), Rhythm disturbances 15 (7.5%), Infective endocarditis 5(2.5%) were also present along with co-morbid conditions like coronary artery disease (1.5% and systemic hypertension (2.5%). Dyspnea on exertion (59.5%) followed by cyanosis (41%) were the most common presenting complaints. The most common reason for hospital admission was cardiac signs and symptoms (19.5%) followed by cardiac catheterization (10.5%). CONCLUSION: The ASD, TOF, VSD and PDA remain the most common CHD in descending order while pulmonary hypertension, Eisenmenger, heart failure, arrhythmias, infective endocarditis and stroke were the common complications of CHD at this particular age.

Physiological pacing in young patients with complex congenital heart defects.

AIM: Young patients with operated complex congenital heart defects (CHD) often develop sinus node dysfunction (SND) requiring permanent pacing with rate-responsive function. Activity-driven sensors cannot account for nonmovement stress and cannot modulate heart rate physiologically. Closed Loop Stimulation (CLS, Biotronik, Berlin, Germany) is a physiological rate-responsive pacemaker based on the indirect measure of ventricular contractility. No data are available on the effects of such pacing strategy in young patients. METHODS: We report a series of nine patients with CHD and SND who underwent single-chamber CLS-atrial pacing with endocardial or epicardial lead. During the first 30 days, the pacemaker was programmed in AAI pacing mode and then was switched to CLS-atrial pacing mode. An in-hospital control was scheduled 1-2 months later to evaluate the CLS response to neurovegetative stresses (i.e., nonmovement stress [Stroop color test, handgrip] and exercise stress test) and Holter monitor. CLS pacing was compared with rate-responsive accelerometer-driven pacing (AAIR). RESULTS: At telemetric interrogation, CLS pacing showed a more physiological pattern of 24-h heart rate trends than accelerometer sensors. The data obtained during nonmovement/exercise stress demonstrated a physiological increase in the pacing rate with CLS, in synergy with spontaneous events. The accelerometer sensor histogram, during nonmovement stress, showed a "nonresponse" behavior (only lower rate events), and during exercise test showed most events in lower rate range. Holter monitoring showed increase of average and maximum heart rate compared with AAIR. CONCLUSION: In young CHD patients, endocardial/epicardial CLS-atrial pacing demonstrated a physiological response of heart rate to neurovegetative and physical stresses.

Postcardiotomy refractory ventricular fibrillation: rescue using veno-arterial extracorporeal membrane oxygenation.

We present a case of 7-hour ventricular fibrillation with successful use of veno-arterial extracorporeal membrane oxygenation as a bridge to recovery in a 30-year-old patient with grown-up congenital heart disease who underwent pulmonary valve replacement.

Timing of Pulmonary Valve Replacement: How Much Can the Right Ventricle Dilate Before it Looses Its Remodeling Potential?

Congenital heart disease patients that develop secondary pulmonary regurgitation require a pulmonary valve replacement (PVR) in their follow-up. The indications for PVR in asymptomatic patients are debated. Most guidelines consider a RV end-diastolic volume (RVEDV) over 150 ml/m(2) as an indication for PVR. We analyzed clinical, echocardiographic and MRI variables of patients that underwent a surgical PVR between September 2006 and February 2013. The included patients were asymptomatic, without pulmonary stenosis and with both pre- and post-surgery MRI. Thirty-five patients (74.3 % males) were included. Mean age at PVR was 25.8 years (SD = 7.18), and weight was 64.5 Kg (SD = 12.03). The main diagnosis was tetralogy of Fallot (n = 28), pulmonary atresia (n = 2), primary pulmonary regurgitation (n = 2) and pulmonary regurgitation after percutaneous treatment (n = 2). The maximal RVEDV pre-PVR was 267 ml/m(2), and right ventricular end-systolic volume (RVESV) was 183 ml/m(2). RV size and function were established by MRI: Pre-PVR Post-PVR p RVEDV (ml/m(2)) 162 (SD = 39.1) 94 (SD = 23.6) <0.001 RVESV (ml/m(2)) 87 (SD = 28.9) 44 (SD = 15.7) <0.001 RVEF 44.8 % (SD = 8.17) 52 % (SD = 9.9) <0.001 Patients with a RVEDV under 170 ml/m(2) combined with a RVESV under 90 ml/m(2) had a favorable RV remodeling, defined as RVEDV under 110 ml/m(2) (sensitivity 87.5 %), RVESV under 55 ml/m(2) (sensitivity 100 %) and RVEF over 50 % (sensitivity 100 %). When deciding the optimal PVR timing in asymptomatic patients, both RVEDV and RVESV should be considered. Our results suggest that higher volumes than used in the clinical practice can achieve a good remodeling. Therefore, PVR could be performed later in the follow-up reducing the number of cardiac interventions.

Valve-sparing double root replacement after the Ross procedure.

An inherent limitation of the Ross procedure is long-term two valve disease which will require repetitive reintervention. In this case, a 31-year-old man who had underwent Ross operation due to congenital bicuspid valve 20 years before, underwent double root replacement: valve sparing root reimplantation concomitant with the right ventricular outflow tract (RVOT) reconstruction with a bioprosthesis for severe RVOT stenosis. Although the diameter of autograft root was 42 mm and aortic insufficiency was mild, we added root surgery due to concerns regarding autograft root dilation in response to left ventricular volume load after RVOT reconstruction. The postoperative echocardiogram showed minimal aortic valve regurgitation and normal blood flow in the RVOT, and he was discharged from the hospital on the 17th day after the surgery. In this report, we present the outcomes of Valve-sparing double root replacement following Ross surgery.

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.

Inadvertent left ventricular placement of ICD lead through the left subclavian artery right positioned in a patient with situs viscerum inversus and Kartagener syndrome.

Inadvertent placement of pacemaker and implantable cardioverter-defibrillator (ICD) leads in the left ventricle is a rare but well-recognized complication of device implantation. We report a case of an unicameral ICD lead inadvertently placed through the left subclavian artery right positioned, across the aortic valve into the left ventricle, in a patient with situs viscerum inversus. A transthoracic echocardiogram about a month after the procedure showed an unusual course of the lead. The lead was successfully removed without complications or sequelae. .

Risk evaluation in adult congenital heart surgery: analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database risk models on data from the European Congenital Heart Surgeons Association Congenital Database.

OBJECTIVES: We sought to evaluate the predictive power of the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) mortality score and the adult congenital heart surgery (ACHS) mortality score for the adults undergoing congenital heart operations entered into the European Congenital Heart Surgeons Association (ECHSA) database. METHODS: The data set comprised 17 662 major operations performed between 1997 and 2019, on patients 18 years of age or older, in European centres participating in the ECHSA database. Each operation was assigned a STAT mortality score and category and an ACHS mortality score. Operative mortality was based on the 30-day status and on the status at hospital discharge. The discriminatory power of the STAT and ACHS scores was assessed using the area under the receiver operating characteristic curve (c-index). RESULTS: A total of 17 214 (97.46%) operations were assigned ACHS scores. The 3 most frequent primary procedures were closure of the atrial septal defect (19.0%), aortic valve replacement (8.8%) and non-valve-sparing aortic root replacement (6.1%). Operative mortality for ACHS-coded operations was 2.07%. The procedures with the highest mortality were atrial septal defect creation/enlargement (19.0%), lung transplantation (18.8%) and heart transplantation (18.2%). A total of 17 638 (99.86%) operations were assigned a STAT score and category. The operative mortality for STAT-coded operations was 2.27%. The c-index for mortality was 0.720 for the STAT mortality score and 0.701 for the ACHS score. CONCLUSIONS: The ACHS mortality score and the STAT mortality score reached similar, moderate predictive power in adult patients undergoing congenital heart surgery in ECHSA database.

German Heart Centre Munich-45 years of surgery in adults with congenital heart defects: from primary corrections of septal defects and coarctation to complex reoperations.

BACKGROUND: Our historical overview in the form of a cohort study aimed to describe the changes in the cardiac operations in adults with congenital heart disease (ACHD), over 45 years in the German Heart Centre in Munich. METHODS: The study population comprised all consecutive patients aged 18 or more, who underwent surgery for congenital heart disease (CHD) at the German Heart Centre Munich, between 1974 and 2018. Operations were coded according to the Adult Congenital Heart Surgery (ACHS) score. Two-tailed chi-square test was used for testing on differences in the frequency of procedural groups between the decades. RESULTS: During the examined 45-year period, 2,882 operations were performed on ACHD. The number of operations per year increased with a correlation coefficient r=0.533, P<0.001. Overall operative mortality was 3.2%. There was no significant difference in mortality over the decades. The percentage of primary CHD diagnosis being the indication for the operation was 99% in the 1970s and decreased significantly to 56% in the 2010s, P<0.001. There was a significant decrease in the relative frequency of atrial septal defect closures from 45% in the 1970s and 1980s to 9% in 2010s (P<0.001). Coarctation repair made up 15% of all operations in the 1970s, then dropped significantly to 1% in the 2010s (P<0.001). The percentage of reoperations increased significantly from 7% in the 1970s to 50% in 2010s (P<0.001). CONCLUSIONS: The spectrum of cardiac surgery in ACHD changed significantly over the last 45 years, from primary repair of septal defects and coarctation of the aorta in the 1970s to complex reoperations in the 2010s.

Palliative care for people living with cardiac disease.

Many cardiovascular diseases lead to heart failure, which is a progressive syndrome causing significant distress and limiting the quality of life, despite optimal cardiologic treatment. It is estimated that about 26 000 people in Poland suffer from advanced heart failure, and this number is growing. That is why palliative care (PC) dedicated to people living with end­stage cardiac diseases should be urgently implemented in Poland. Well­organized PC may not only relieve symptoms and improve quality of life in people living with cardiac diseases not responding to treatment but also support patients and their families during the dying process. Palliative care in patients with cardiac diseases should be continued during the end-of-life period. It should be implemented regardless of prognosis, and adjusted to patients' needs. Two approaches to PC are presented in this expert opinion. The first one (generic) is provided by all medical professionals incorporating PC principles into the usual patient care. The second approach, namely, specialized PC, is ensured by a multiprofessional team or at least a PC specialist who received appropriate training in PC. The model of needs-based (not prognosis-based) implementation of PC is discussed in this paper. Symptom control, support in decision-making, and sensitive, open communication are considered integral elements of PC interventions. Medical professionals developing PC in Poland should think about groups of patients with special needs like those with valvular heart disease, grown­up congenital heart disease, and pulmonary arterial hypertension, as well as elderly people. This consensus document presents main recommendations for future PC organization in Poland. Among others, we suggest changing the Polish National Health Fund reimbursement rules regarding PC and improving cardiologist education on PC.

Italian survey on cardiac surgery for adults with congenital heart disease: which surgery, where and by whom?

OBJECTIVES: The population of ageing adults with congenital heart disease (ACHD) is increasing; surgery in these patients presents major difficulties in management. A great debate has developed about whether these patients should be cared for at an adult or paediatric hospital and by an acquired or congenital cardiac surgeon. We analysed data of the surgical treatment of ACHD from the Italian cardiac surgery centres in 2016, focusing on the type of surgery performed, where these patients were operated on and by whom. METHODS: Ninety-two Italian cardiac surgery centres were contacted and 70 centres participated in this study. We collected data on the types of cardiac operations performed in congenital heart defect patients older than 18 years. In 2016, a total of 913 patients with ACHD were operated on: 440 by congenital cardiac surgeons (group I) in centres with paediatric and adult cardiac surgery units, and 473 by adult cardiac surgeons (group II) in centres with exclusively adult cardiac surgery units. RESULTS: Pathologies of the right ventricular outflow tract were the most frequent diseases treated in group I and pathologies of the left ventricular outflow tract in group II. Group I included more complex and heterogeneous cases than group II. Surgery for ACHD represented 12% of the activity of congenital cardiac surgeons and only 1% of the activity of adult cardiac surgeons. CONCLUSIONS: In Italy, ACHD patients are operated on both by congenital and adult cardiac surgeons. Congenital cardiac surgeons working in centres with both paediatric and adult cardiac surgery are more involved with ACHD patients and with more complex cases.

Amniotic Fluid Embolism in a Grown-Up Congenital Heart Disease Patient.

Amniotic fluid embolism (AFE) is a rare but potentially lethal obstetric condition affecting women during labor, delivery, or also in the immediate postpartum period, when amniotic fluid, fetal cells, hair, or other debris could enter the maternal circulation. We present the first case of AFE described in literature in a 33-year-old patient with a complex history of congenital heart disease, where the crucial points for successful course were the prompt recognition and treatment of the disease and the use, for the first time, as diagnostic tool for AFE, of right ventricular free-wall longitudinal speckle-tracking strain.

Small atrial septal defects are associated with psychiatric diagnoses, emotional distress, and lower educational levels.

OBJECTIVE: For the first time, we wish to assess the psychiatric burden in adult patients living with small, unrepaired atrial septal defects (ASD) using register-based data, combined with self-reported measures on levels emotional distress and educational status. DESIGN: A descriptive study using both the unique Danish registries and validated psychiatric questionnaires and scales, including: The Symptom Checklist, Whiteley-7, and Brief Illness Perception Questionnaire. PATIENTS: Adult patients with small, unrepaired ASD, diagnosed between 1953 and 2011. OUTCOME MEASURES: Number of register-based psychiatric diagnoses. Additionally, symptoms of anxiety, depression, somatization, health anxiety, illness perception, and levels of educational attainment compared to age- and gender-matched individuals from the reference group. RESULTS: We identified 723 Danish patients with a small, unrepaired ASD. Approximately 17% of the patients eligible for inclusion had one or more psychiatric diagnoses. The most common diagnoses were neurotic, stress-related, and somatoform disorders. A total of 140 patients (mean age 33 years) was included for examination of their mental health using psychiatric questionnaires and scales. Patients with small ASD had higher scores of depression and anxiety when compared to the reference group (0.57 ± 0.67 vs 0.39 ± 0.52; P < .001). Furthermore, patients reported more symptoms of somatization (0.51 ± 0.48 vs 0.34 ± 0.39; P < .001). Finally, a higher percentage of patients with an unrepaired ASD had no education beyond high school when compared to the reference group (33.3% vs 14.3%; P < .001) and a smaller proportion of patients had completed a short-cycle higher education (6.5% vs 16.1%; P = .002). CONCLUSION: Patients with a small, unrepaired ASD in adult life have more symptoms of anxiety, depression, and somatization compared to the reference group. Furthermore, fewer patients had no education beyond high school. These results support the guidelines that adults with small ASD warrants regular lifelong follow-up in specialized clinics aware of not only adverse somatic outcome but also rates of depression and anxiety.

Never too Grown-Up for a Congenital Heart Disease: Diagnosis of Transitional Atrioventricular Canal in a 50-Year-Old Male.

Transitional atrioventricular (AV) septal defects are uncommon congenital heart defects, and diagnosis is usually made in childhood. We present the case of intermediate AV canal diagnosed in a man referring to cardiological examination for the first time in his life at the age of fifty for exertional dyspnea. The absence of medical examination or execution of electrocardiogram or echocardiogram in childhood or in youth and the very late appearance of symptoms lead to a late diagnosis of this congenital heart disease (CHD). This case underlines the importance of including CHD in the differential diagnosis of symptoms such as chronic dyspnea, also in adulthood.

Heart Failure in Adult Patients with Congenital Heart Disease.

Adult patients with congenital heart disease are a complex population with a variety of pathophysiologic conditions based on the anatomy and type of surgery or intervention performed, usually during the first years of life. Nowadays, the majority of patients survive childhood and present for a number of noncardiac surgeries or interventions needing appropriate perioperative management. Heart failure is a major contributing factor to perioperative morbidity and mortality. In this review, we present an overview of the most common types of adult patients with congenital heart disease and actual knowledge on therapy and specific risks in this challenging patient population.

Results for tricuspid valve surgery in adults with congenital heart disease other than Ebstein's anomaly†.

OBJECTIVES: Tricuspid valve (TV) surgery in the adult with congenital heart disease (ACHD) is a frequently performed procedure. The aim of this study was to analyse postoperative and medium-term outcomes. METHODS: We conducted a single-centre retrospective study of patients with ACHD who underwent TV surgery (January 2000-December 2016); patients with Ebstein's anomalies were excluded. Operative and clinical records were reviewed. Outcomes considered were survival, grade of insufficiency/stenosis and TV reoperation at follow-up. RESULTS: A total of 128 patients with ACHD had TV surgery for functional regurgitation (n = 95), dysplasia (n = 23) and systemic TV (n = 10). Median age was 40.8 years [interquartile range (IQR) 25.3]; 55.5% were men. Preoperative regurgitation was classified as mild (n = 8), moderate (n = 47) and severe (n = 70). The TV was repaired in 109 as follows: ring annuloplasty (n = 43), de Vega annuloplasty (n = 29), Wooler annuloplasty (n = 13), commissural plasty (n = 9), Kay annuloplasty (n = 7) and others (n = 8). The TV was replaced in 19 patients with biological (n = 10) and mechanical (n = 9) prostheses. The median hospital stay was 12 days (IQR 10). The overall mortality rate was 8.6% (n = 11): 2 hospital deaths (1.6%) and 9 late deaths. Survival was 93% [95% confidence interval (CI) 85-97%] at 5 years and 83% (95% CI 70-91%) at 10 years. The median follow-up period was 4.95 years (IQR 7.7) with 1 TV reoperation. Echocardiographic assessment showed ≥moderate regurgitation in 34 (34.3%) patients. Suture plasty had a significantly higher incidence of TV regurgitation ≥moderate compared to ring annuloplasty (48.9% vs 26.3%; P = 0.033). CONCLUSIONS: TV surgery in the ACHD is frequently associated with other main procedures. Stabilizing the TV annulus with a prosthetic ring guarantees lower recurrence of moderate to severe regurgitation compared to suture plasty repair.

Adult congenital heart disease nurse coordination: Essential skills and role in optimizing team-based care a position statement from the International Society for Adult Congenital Heart Disease (ISACHD).

BACKGROUND: Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with congenital heart disease (CHD) worldwide. Among ISACHD's objectives is to "promote a holistic team-based approach to the care of the adult with CHD that is comprehensive, patient-centered, and interdisciplinary" (http://www.isachd.org). This emphasis on team-based care reflects the fact that adults with CHD constitute a heterogeneous population with a wide spectrum of disease complexity, frequent association with other organ involvement, and varied co-morbidities and psychosocial issues. METHODS: Recognizing the vital role of the adult CHD (ACHD) nurse coordinator (ACHD-NC) in optimizing team-based care, ISACHD established a task force to elucidate and provide guidance on the roles and responsibilities of the ACHD-NC. Acknowledging that nursing roles can vary widely from region to region based on factors such as credentials, scopes of practice, regulations, and local culture and tradition, an international panel was assembled with experts from North America, Europe, East Asia, and Oceania. The writing committee was tasked with reviewing key aspects of the ACHD-NC's role in team-based ACHD care. RESULTS/CONCLUSION: The resulting ISACHD position statement addresses the ACHD-NC's role and skills required in organizing, coordinating, and facilitating the care of adults with CHD, holistic assessment of the ACHD patient, patient education and counseling, and support for self-care management and self-advocacy.

Diagnostic value of the six-minute walk test (6MWT) in grown-up congenital heart disease (GUCH): Comparison with clinical status and functional exercise capacity.

BACKGROUND: Exercise testing for the assessment of functional capacity plays an important role in long-term follow-up of GUCH patients. CPX is the favored modality for decision-making recommended in the current guidelines. In contrast to this complex method, the 6 MWT is a simple, easy-to-perform, safe, and commonly available exercise test. Although well-established in various cardiopulmonary diseases, the diagnostic impact of the 6 MWT in GUCH patients is not known so far. METHODS: 102 GUCH patients were evaluated by 6 MWT and CPX simultaneously. Clinical symptoms were assessed, according to the NYHA classification. Additionally, an echocardiography study, and selected cardiac blood tests (N-terminal pro-brain natriuretic peptide (NT-proBNP), high-sensitive Troponin T) were performed. RESULTS: Ranges of six-minute walk distance (6 MWD) and peak oxygen consumption (peakVO2) were 116-765 m and 6.4-36.2 ml/kg/min, respectively. 6 MWD and peakVO2 showed a close correlation (r=0.72, 95% CI, 0.63 to 0.79). Patients with a peakVO2 of ≤ 15.5 ml/kg/min were excellently identified by 6 MWT (c-value=0.82). A cut-off value of 482 m was optimal to predict reduced peakVO2. In multivariate regression analysis, 6 MWD and NYHA class were identified as relevant predictors of peakVO2. In subgroup analysis, Eisenmenger patients achieved the shortest 6 MWD (280, SD 178 m). CONCLUSION: In our study population of GUCH patients, the 6 MWD shows a close correlation to peakVO2, and an excellent prediction of reduced peakVO2. Thus, it seems to be an easy-to-perform and reliable screening parameter to evaluate functional capacity of these patients (Controlled Clinical Trials number, NCT02193243).