Paul Clift

Percutaneous device closure of a pseudoaneurysm of the left ventricular wall

The percutaneous device closure of a left ventricular pseudoaneurysm is described in a 60 year old man with a history of myocardial infarction complicated by ventricular tachycardia and left ventricular aneurysm treated by coronary artery bypass grafting and aneursymectomy with ventricular tachycardia ablation. He subsequently developed a vast pseudoaneurysm of the left ventricle with New York Heart Association functional class II heart failure symptoms. The selection of the approach and type of device used to close the neck of the pseudoaneurysm are discussed.

Percutaneous Device Closure of Paravalvular Leak: Combined Experience from the United Kingdom and Ireland

Background: Paravalvular leak (PVL) occurs in 5% to 17% of patients following surgical valve replacement. Percutaneous device closure represents an alternative to repeat surgery. Methods: All UK and Ireland centers undertaking percutaneous PVL closure submitted data to the UK PVL Registry. Data were analyzed for association with death and major adverse cardiovascular events (MACE) at follow-up. Results: Three hundred eight PVL closure procedures were attempted in 259 patients in 20 centers (2004–2015). Patient age was 67±13 years; 28% were female. The main indications for closure were heart failure (80%) and hemolysis (16%). Devices were successfully implanted in 91% of patients, via radial (7%), femoral arterial (52%), femoral venous (33%), and apical (7%) approaches. Nineteen percent of patients required repeat procedures. The target valve was mitral (44%), aortic (48%), both (2%), pulmonic (0.4%), or transcatheter aortic valve replacement (5%). Preprocedural leak was severe (61%), moderate (34%), or mild (5.7%) and was multiple in 37%. PVL improved postprocedure ( P <0.001) and was none (33.3%), mild (41.4%), moderate (18.6%), or severe (6.7%) at last follow-up. Mean New York Heart Association class improved from 2.7±0.8 preprocedure to 1.6±0.8 ( P <0.001) after a median follow-up of 110 (7–452) days. Hospital mortality was 2.9% (elective), 6.8% (in-hospital urgent), and 50% (emergency) ( P <0.001). MACE during follow-up included death (16%), valve surgery (6%), late device embolization (0.4%), and new hemolysis requiring transfusion (1.6%). Mitral PVL was associated with higher MACE (hazard ratio [HR], 1.83; P =0.011). Factors independently associated with death were the degree of persisting leak (HR, 2.87; P =0.037), New York Heart Association class (HR, 2.00; P =0.015) at follow-up and baseline creatinine (HR, 8.19; P =0.001). The only factor independently associated with MACE was the degree of persisting leak at follow-up (HR, 3.01; P =0.002). Conclusion: Percutaneous closure of PVL is an effective procedure that improves PVL severity and symptoms. Severity of persisting leak at follow-up is independently associated with both MACE and death. Percutaneous closure should be considered as an alternative to repeat surgery. # Clinical Perspectives {#article-title-21}Background: Paravalvular leak (PVL) occurs in 5% to 17% of patients following surgical valve replacement. Percutaneous device closure represents an alternative to repeat surgery. Methods: All UK and Ireland centers undertaking percutaneous PVL closure submitted data to the UK PVL Registry. Data were analyzed for association with death and major adverse cardiovascular events (MACE) at follow-up. Results: Three hundred eight PVL closure procedures were attempted in 259 patients in 20 centers (2004–2015). Patient age was 67±13 years; 28% were female. The main indications for closure were heart failure (80%) and hemolysis (16%). Devices were successfully implanted in 91% of patients, via radial (7%), femoral arterial (52%), femoral venous (33%), and apical (7%) approaches. Nineteen percent of patients required repeat procedures. The target valve was mitral (44%), aortic (48%), both (2%), pulmonic (0.4%), or transcatheter aortic valve replacement (5%). Preprocedural leak was severe (61%), moderate (34%), or mild (5.7%) and was multiple in 37%. PVL improved postprocedure (P<0.001) and was none (33.3%), mild (41.4%), moderate (18.6%), or severe (6.7%) at last follow-up. Mean New York Heart Association class improved from 2.7±0.8 preprocedure to 1.6±0.8 (P<0.001) after a median follow-up of 110 (7–452) days. Hospital mortality was 2.9% (elective), 6.8% (in-hospital urgent), and 50% (emergency) (P<0.001). MACE during follow-up included death (16%), valve surgery (6%), late device embolization (0.4%), and new hemolysis requiring transfusion (1.6%). Mitral PVL was associated with higher MACE (hazard ratio [HR], 1.83; P=0.011). Factors independently associated with death were the degree of persisting leak (HR, 2.87; P=0.037), New York Heart Association class (HR, 2.00; P=0.015) at follow-up and baseline creatinine (HR, 8.19; P=0.001). The only factor independently associated with MACE was the degree of persisting leak at follow-up (HR, 3.01; P=0.002). Conclusion: Percutaneous closure of PVL is an effective procedure that improves PVL severity and symptoms. Severity of persisting leak at follow-up is independently associated with both MACE and death. Percutaneous closure should be considered as an alternative to repeat surgery.

The Safety and Effects of Bosentan in Patients with a Fontan Circulation

OBJECTIVE   Adult patients with a Fontan circulation tend to have diminished exercise capacity. The principal objective of this study was to investigate the safety of the endothelin receptor antagonist bosentan in Fontan patients, and, secondarily, to assess effects on cardiovascular performance, New York Heart Association functional classification (NYHA FC), and ventricular function. DESIGN   A 6-month prospective, single-center, pilot, safety study of bosentan in Fontan patients. Setting.  Adult Congenital Heart Disease referral center. PATIENTS   All patients ≥18 years old with a Fontan circulation and in NYHA FC ≥II were invited to enroll. Interventions.  Patients started on 62.5 mg bid of bosentan, uptitrating to 125 mg bid after 2 weeks. OUTCOME MEASURES   Safety was assessed by the incidence of anticipated and unanticipated adverse events during the 6-month study period; specifically those relating to hepatic, renal, or hematological dysfunction as measured by monthly blood tests. Other outcome measures included cardiopulmonary exercise test, 6-minute walk distance test, Borg dyspnea index, NYHA FC, and ventricular function parameters using transthoracic echocardiography. RESULTS   Of the eight patients enrolled, six completed the study. Two patients withdrew from the study (one for non-trial related reasons, one due to adverse events). No clinically significant adverse events relating to bosentan therapy occurred during this study and, in particular, no significant abnormalities in hepatic function tests were observed. Three patients reported transient adverse events. Improvements in NYHA FC and systolic ventricular function were observed after 6 months of bosentan treatment. CONCLUSIONS   The small number of patients with a Fontan circulation in our study was able to tolerate bosentan for 6 months. The safety and tolerability of bosentan in a larger patient population remains unknown. The results presented here justify further investigation in larger studies.

Long-term Outcome Following Pregnancy in Women with a Systemic Right Ventricle: Is the Deterioration due to Pregnancy or a Consequence of Time?

INTRODUCTION The right ventricle (RV) supports the systemic circulation in patients who have had an intraatrial repair of transposition of the great arteries or have congenitally corrected transposition. There is concern about the ability of a systemic RV to support the additional volume load of pregnancy, and previous studies have reported deterioration in RV function following pregnancy. However, conditions with a systemic RV are also associated with progressive RV dysfunction over time. To date, no study has examined whether the deterioration associated with pregnancy is due to the physiological changes of pregnancy itself, or is part of the known deterioration that occurs with time in these patients. METHODS Women who had undergone pregnancy under the care of the Adult Congenital Heart Disease Unit at the Queen Elizabeth Hospital were retrospectively identified and matched to separate male and nulliparous female controls. Functional status (New York Health Association [NYHA]), RV function, and systemic atrioventricular valve regurgitation were recorded for each group at baseline, postpregnancy (or at 1 year for control groups) and at latest follow-up. RESULTS Eighteen women had 31 pregnancies (range 1-4) resulting in 32 live births. There were no maternal but one neonatal death. At baseline, there was no significant difference in NYHA class or RV function between pregnancy and control groups. In postpregnancy, there was a significant deterioration in the pregnant group alone for both NYHA class (P = 0.004) and RV function (P = 0.02). At latest follow-up, there was a significant deterioration in RV function in all three groups. There was still a reduction from baseline in NYHA of women who had undergone pregnancy (P = 0.014), which again was not seen in the controls groups. CONCLUSION This study suggests that pregnancy is associated with a premature deterioration in RV function in women with a systemic RV. These women are also more symptomatic, with a greater reduction in functional class compared with patients with a systemic RV who do not undergo pregnancy. This study will allow this cohort of women to be more accurately counseled as to the potential long-term risks of pregnancy.

The role of echocardiography in diagnosis, monitoring closure and post-procedural assessment of patent foramen ovale

Patent foramen ovale (PFO) is common, with a probe-patent PFO present in 15-35% of the general population. Patent foramen ovale has been implicated in the aetiology of a number of different pathologies, including cryptogenic stroke, decompression sickness in divers, platypnea orthodeoxia, and migraine with aura. Cardiac ultrasound has a major role not only in the diagnosis of PFO but also in monitoring subsequent therapeutic intervention and in the post-procedural assessment of patients following percutaneous closure. The aim of this review was to outline the data regarding the role of echocardiography in diagnosis, during monitoring and post-procedural assessment so as to provide practical advice to minimize error and optimize patient outcomes. The review will seek to outline the limitations of the available techniques and factors that should be taken into account during percutaneous device closure.

Patent foramen ovale closure with the Gore septal occluder: initial UK experience.

To report procedural outcome and short‐term follow‐up data for the Gore septal occluder (GSO), a new device for closure of patent foramen ovale (PFO).

Permanent trans-venous pacing in an extra-cardiac Fontan circulation

AIMS In patients with an extra-cardiac Fontan circulation, there is no direct access to the heart. The insertion of a permanent pacemaker requires surgery to insert epicardial pacing wires. We present the implantation of a permanent endocardial pacing lead from the superior vena cava (SVC) into the atrium via direct passage from the right pulmonary artery (RPA). METHODS AND RESULTS A permanent pacing lead was passed directly from the SVC to the RPA and then into the atrial mass. Direct passage from the RPA (attached directly to the right SVC) into the atrial mass was achieved using a trans-septal puncture needle. CONCLUSION This novel technique is an alternative to epicardial pacing in patients with an extra-cardiac Fontan circulation, thus avoiding the need for surgical intervention. It may also be applied to gain access to the atrial mass for arrhythmia ablation therapy.

Femoral Vascular Access Complications in Adult Congenital Heart Disease Patients: Audit from a Single Tertiary Center

OBJECTIVE To determine the rate of vascular access complications in patients with adult congenital heart disease (ACHD). BACKGROUND Complications of femoral access following coronary angiography or percutaneous coronary intervention have been studied extensively, but the complication rate following catheterization and intervention in ACHD patients is poorly documented. DESIGN, SETTING, AND OUTCOME MEASURES: We present a retrospective audit of vascular access complications in a large tertiary ACHD center over a 12-month period. Complications were defined as any clinically significant hematoma, pseudoaneurysm, arteriovenous fistula, or bleeding resulting in the need for imaging, transfusion, vascular or radiological intervention, or delayed discharge. RESULTS Of 197 procedures (102 interventions and 95 cardiac catheterizations), a complication rate of 3.6% was identified, comparable to that of coronary angiography and percutaneous coronary intervention. The main complications were femoral artery pseudoaneurysm and hematoma resulting in delayed discharge by a mean of 2(2/3) days (range 1-4 days). Predictors of risk for vascular complications include female sex, history of diabetes, and anticoagulation; larger sheath sizes and obesity were not associated with higher complication rate. CONCLUSIONS Adult congenital heart disease patients represent a unique and ever-growing population with a higher incidence of catheterization as children, surgical cut-down scars and anatomical variants. We present a low incidence of femoral access complications in interventional and diagnostic procedures in a large series of ACHD patients over a 12-month period. Patients with risk factors for vascular complications may be considered for device closure of the venous access site.

Outcomes of Patients With Hypoplastic Left Heart Syndrome Reaching Adulthood After Fontan Palliation: Multicenter Study

Hypoplastic left heart syndrome (HLHS) is a high-risk condition among the wider spectrum of single-ventricle physiology lesions, and few survivors have reached adulthood.1 As HLHS is one of the most common diagnoses for children undergoing Fontan palliation,2 identification of risk factors for adverse outcomes in this emerging population is of importance. We report results from a multicenter observational study that characterizes adults with Fontan palliation for underlying HLHS at entry into adulthood and defines factors associated with adverse outcomes. Adults (≥18 years by July 2014) with HLHS were studied. The diagnosis of HLHS was restricted to patients with mitral stenosis or atresia, aortic stenosis or atresia, and a diminutive left ventricle. Excluded were patients with other forms of dominant right ventricular lesions with univentricular physiology managed with a Norwood procedure (ie, unbalanced atrioventricular septal defect or double-outlet right ventricle) or those with heart transplantation. Available pediatric and adult cardiovascular data were reviewed. The primary end point (Fontan-specific major adverse cardiovascular event) was defined as death, transplantation listing, hospitalization (>24 hours) for heart failure requiring intravenous diuretics, sustained ventricular …

Adult congenital heart disease interventions: Recommendations from a Joint Working Group of the British Congenital Cardiac Association, British Cardiovascular Intervention Society, and the British Cardiovascular Society

In order to optimise care of the adult patients with complex congenital heart disease, there is a need to develop recommendations for interventions. This document is the work of representatives of the three relevant societies and provides recommendations for institutions and operators performing cardiac interventions in these patients.