Adult Congenital Disease Research Articles

2019 AATS/ACC/ASE/SCAI/STS expert consensus systems of care document: A proposal to optimize care for patients with valvular heart disease: A Joint Report of the American Association for Thoracic Surgery, American College of Cardiology, American Society of Echocardiography, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons.

This statement was commissioned as a Multisociety Expert Consensus Systems of Care Document by the American Association for Thoracic Surgery (AATS), American College of Cardiology (ACC), American Society of Echocardiography (ASE), Society for Cardiovascular Angiography and Interventions (SCAI), and

Echocardiographic evaluation of cardiac function after cancer chemotherapy.

Progress in cancer therapy has led to improved prognosis of patients with cancer and thus to a continuous rise of cancer survivors. However, it has simultaneously increased cardiovascular morbidity and mortality rates due to direct and/or indirect side effects of anticancer treatment. Similar to the rapid rise of patients with adult congenital disease, the number of patients suffering or at risk of cardiotoxicity has been steeply increasing and getting an emerging issue. Among the many facets of chemotherapy-induced cardiovascular toxicity, this review attempts to summarize echocardiographic evaluation of cardiac function after cancer chemotherapy by reviewing the definition, risk factors, brief history, limitation of left ventricular ejection fraction and myocardial strain imaging, as well as the limitations of this technique.

Impact of Isolated Tricuspid Valve Repair on Right Ventricular Remodelling in an Adult Congenital Heart Disease Population.

Surgical repair of isolated congenital tricuspid valve (TV) disease is rare with no well-defined indication and outcomes. Moreover, the role of right ventricle (RV) in this context has not yet been investigated. We sought to assess the impact of congenital TV repair on cardiac remodelling and clinical-functional status and the importance of the RV function in an adult congenital heart disease (ACHD) population. From January 2005 to December 2015, 304 patients underwent TV surgery in our centre. Of these, 27 (ACHD) patients had isolated TV repair. Patients were evaluated with preoperative and postoperative transthoracic echocardiogram. Survival rate has been investigated with a mean clinical follow-up (FU) of 3.7 ± 2.3 years, whereas the mean echocardiographic FU was 2.9 ± 1.8 years. The clinical and functional status of patients showed a statistically significant improvement after the surgical repair in terms of New York Heart Association class (66.7 vs 7.4%; p < 0.01), clinical signs of heart failure (29.6 vs 7.4%; p < 0.01), and left ventricular function (14.8 vs 7.4%; p < 0.01). The RV and right atrium diameter were significantly reduced after surgery (5.15 ± 1.21 vs 4.32 ± 1.16; p < 0.01) and (44.7 ± 16.7 vs 26.7 ± 9.2; p < 0.01), respectively. The degree of postoperative pulmonary hypertension was also significantly reduced (40.7 vs 7.4%; p < 0.01). The survival rate was 96.3% at 1 year and 93.7% at 5 years. One patient (3.7%) had early failure of the tricuspid repair requiring a reoperation. Isolated TV repair for adult congenital disease significantly improved patients' clinical and functional status and allowed right ventricular remodelling and functional improvement.

P25 Stroke risk from atrial fibrillation in repaired tetralogy of fallot

Background Atrial fibrillation (AF) has a prevalence of 1.6% in the UK and significantly increases the risk of developing a stroke. Adults with congenital heart disease have an increased risk of developing arrhythmias, a leading cause of morbidity and mortality in these patients. CHADSVASC scoring system is a recommended tool for assessing thromboembolic risk in AF for the general population; its role in adult congenital heart disease is still uncertain. Method All patients with repaired Tetralogy of Fallot (TOF) and atrial fibrillation were identified from our database and their notes were retrospectively reviewed. Results 35 of the 456 (7.7%) patients with repaired TOF under regular follow-up at a large quaternary Specialist Adult Congenital Disease Centre were identified as having AF, mean age 56.5(+/-12.1) years, range 27–77 years, 19 male (54.3%). The average CHADSVASC score was 1.83 (range 0–4). Most were anticoagulated; 28 (80%) received warfarin, 3 (8.6%) took a novel anticoagulant (NOAC) but 4 patients were not anticoagulated. The mean age of initial AF diagnosis was 48(+/-13.7) years. 15 (42.9%) patients were diagnosed with AF before the age of 45 years and 7 (20%) before the age of 35 years. Most patients were symptomatic. 5 (14.3%) patients had either a stroke or a transient ischaemic attack. 2 had multiple strokes. 3 of the patients had strokes when they had known AF but were not anticoagulated having CHADVASC scores of 2 or less. 1 patient had 2 strokes in their twenties but was not diagnosed with AF until their 40s. 1 patient suffered a stroke while prescribed rivaroxaban. Conclusion The risk of stroke in TOF appears high. It is unclear whether the CHADSVASC score is useful for risk stratification. This data suggest that anticoagulation should be considered in all patients with TOF and AF, irrespective of CHADSVASC score. The role of NOACs in this population is unclear.

Surgical Closure of Patent Foramen Ovale for Stroke Prevention: Vancouver General Hospital Experience

95 Background and Purpose Patent foramen ovale (PFO) is implicated as a potential cause of stroke, particularly in young patients with otherwise cryptogenic events. The natural history, risk of stroke recurrence and optimal secondary stroke prevention remains uncertain. Therapeutic options include long-term antiplatelet therapy, anti-coagulation therapy, and PFO closure by surgery or device. We report the results of 53 patients treated with surgical closure. Methods and Materials We have followed 53 consecutive surgically treated patients (23 men and 30 female). All patients were evaluated by a stroke neurologist, a cardiologist with expertise in adult congenital disease and a cardiovascular surgeon. Patients who met the following criteria were included: 1) embolic TIA or stroke, 2) PFO or PFO and atrial septal aneurysm (ASA), 3) investigations included cerebral angiography, transesophageal echocardiography, and hypercoagulable studies, 4) presumptive clinical diagnosis of paradoxical embolism with no other etiology detected. Follow up was obtained by clinic visit and standardized telephone questionnaire. Results Prior to surgery 27 patients had stroke and 26 had TIAs; 12 had multiple cerebrovascular events. The mean age at symptom onset was 41.8 ± 9.3 yrs (range 19 to 59). 22 patients had an isolated PFO and 31 had both a PFO and an ASA. Average PFO size measured at surgery was 8.8 ± 7.7 mm. 40 were treated with primary closure, 13 with suture and patch closure. Average post-surgical hospital stay was 4 days. There was no surgical mortality or major morbidity. Minor perioperative morbidity occurred in 13 patients. Average follow up postsurgery was 22.2 ± 17.4 months (range 0.7 to 90.8 months). There were no recurrent strokes and 1 recurrent TIA. Conclusions Surgical closure of PFO can be safely performed with low morbidity and mortality. In this group of carefully selected patients, there have been no recurrent strokes. Further studies are necessary to define high-risk patients for recurrent stroke who may benefit from surgical closure.

Surgical Closure of Patent Foramen Ovale for Stroke Prevention: Vancouver General Hospital Experience

95 Background and Purpose Patent foramen ovale (PFO) is implicated as a potential cause of stroke, particularly in young patients with otherwise cryptogenic events. The natural history, risk of stroke recurrence and optimal secondary stroke prevention remains uncertain. Therapeutic options include long-term antiplatelet therapy, anti-coagulation therapy, and PFO closure by surgery or device. We report the results of 53 patients treated with surgical closure. Methods and Materials We have followed 53 consecutive surgically treated patients (23 men and 30 female). All patients were evaluated by a stroke neurologist, a cardiologist with expertise in adult congenital disease and a cardiovascular surgeon. Patients who met the following criteria were included: 1) embolic TIA or stroke, 2) PFO or PFO and atrial septal aneurysm (ASA), 3) investigations included cerebral angiography, transesophageal echocardiography, and hypercoagulable studies, 4) presumptive clinical diagnosis of paradoxical embolism with no other etiology detected. Follow up was obtained by clinic visit and standardized telephone questionnaire. Results Prior to surgery 27 patients had stroke and 26 had TIAs; 12 had multiple cerebrovascular events. The mean age at symptom onset was 41.8 ± 9.3 yrs (range 19 to 59). 22 patients had an isolated PFO and 31 had both a PFO and an ASA. Average PFO size measured at surgery was 8.8 ± 7.7 mm. 40 were treated with primary closure, 13 with suture and patch closure. Average post-surgical hospital stay was 4 days. There was no surgical mortality or major morbidity. Minor perioperative morbidity occurred in 13 patients. Average follow up postsurgery was 22.2 ± 17.4 months (range 0.7 to 90.8 months). There were no recurrent strokes and 1 recurrent TIA. Conclusions Surgical closure of PFO can be safely performed with low morbidity and mortality. In this group of carefully selected patients, there have been no recurrent strokes. Further studies are necessary to define high-risk patients for recurrent stroke who may benefit from surgical closure.