Nanette Alvarez

Structure and process measures of quality of care in adult congenital heart disease patients: A pan-Canadian study

BACKGROUND There are more adults than children with congenital heart disease. Of over 96,000 ACHD patients in Canada, approximately 50% require ongoing expert care. In spite of published recommendations, data on the quality of care for ACHD patients are lacking. METHODS Survey methodology targeted all Canadian Adult Congenital Heart (CACH) network affiliated ACHD centers. Clinics were asked to prospectively collect outpatient and procedural volumes for 2007. In 2008, centers were surveyed regarding infrastructure, staffing, patient volumes and waiting times. RESULTS All 15 CACH network registered centers responded. The total number of patients followed in ACHD clinics was 21,879 (median per clinic=1132 (IQR: 585, 1816)). Of the total 80 adult and pediatric cardiologists affiliated to an ACHD clinic, only 27% had received formal ACHD training. Waiting times for non-urgent consultations were 4 ± 2 months, and 4 ± 3 months for percutaneous and surgical procedures. These were beyond Canadian recommended targets at 11 sites (73%) for non-urgent consultations, at 8 sites (53%) for percutaneous interventions and 13 sites (87%) for surgery. CONCLUSIONS Of a minimum number of 96,000 ACHD patients in Canada, only 21,879 were being regularly followed in 2007. At most sites waiting times for ACHD services were beyond Canadian recommended targets. In spite of universal health care access, published guidelines for ACHD patient structure and process measures of health care quality are not being met.

Rationale and Design of the Canadian Outcomes Registry Late After Tetralogy of Fallot Repair: The CORRELATE Study

BACKGROUND Chronic hemodynamically relevant pulmonary regurgitation (PR) resulting in important right ventricular dilation and ventricular dysfunction is commonly seen after tetralogy of Fallot (TOF) repair. Late adverse clinical outcomes, including exercise intolerance, arrhythmias, heart failure and/or death accelerate in the third decade of life and are cause for considerable concern. Timing of pulmonary valve replacement (PVR) to address chronic PR is controversial, particularly in asymptomatic individuals, and effect of PVR on clinical measures has not been determined. METHODS Canadian Outcomes Registry Late After Tetralogy of Fallot Repair (CORRELATE) is a prospective, multicentre, Canada-wide cohort study. Candidates will be included if they are ≥ 12 years of age, have had surgically repaired TOF resulting in moderate or severe PR, and are able to undergo cardiovascular magnetic resonance imaging. Enrollment of > 1000 individuals from 15 participating centres (Toronto, Montreal, Quebec City, Sherbrooke, Halifax, Calgary, Edmonton, and Vancouver) is anticipated. Clinical data, health-related quality of life metrics, and adverse outcomes will be entered into a web-based database. A central core lab will analyze all cardiovascular magnetic resonance studies (PR severity, right ventricular volumes, and ventricular function). Major adverse outcomes (sustained ventricular tachycardia and cardiovascular cause of death) will be centrally adjudicated. RESULTS To the best of our knowledge, CORRELATE will be the first prospective pan-Canadian cohort study of congenital heart disease in children and adults. CONCLUSIONS CORRELATE will uniquely link clinical, imaging, and functional data in those with repaired TOF and important PR, thereby enabling critical evaluation of clinically relevant outcomes in those managed conservatively compared with those referred for PVR.

The ten most commonly asked questions about management of congenital heart disease in adults.

Atrial septal defect is often discovered incidentally on echocardiography undertaken for other reasons or as part of a diagnostic work-up for cryptogenic stroke. Many are small and hemodynamically insignificant. The clinical findings associated with an ASD are dependent on the magnitude of the intracardiac shunt and on the pulmonary artery pressures (consequent to the flow and pulmonary vascular resistance). The hallmark of an ASD (whether an ostium secundum ASD, sinus venosus ASD or ostium primum ASD) is fixed splitting of the second heart sound with breathing. If there is a significant intracardiac shunt, a flow murmur may be heard over the pulmonary area because of increased volume of flow (similar to that found in pregnancy). Note that there is no flow murmur across the defect itself because the pressure gradient is low. There may be signs of right ventricular volume overload with a palpable right ventricular lift (a left parasternal heave) and lateral retraction of the apex. If the pulmonary vascular resistance rises, the pulmonary component of the second sound becomes accentuated (and in the later stages, sometimes palpable). Finally, if the pulmonary vascular resistance rises sufficiently, there will be shunt reversal with the development of cyanosis (the Eisenmenger reaction). However, even without the development of excessive pulmonary hypertension, the consequences of longstanding, significant volume loading on the right ventricle may be signs of right ventricular dysfunction with tricuspid regurgitation (and characteristic V-waves in the jugular venous pulse), hepatic congestion, and fluid retention. Atrial arrhythmias, particularly atrial fibrillation, may occur later in life (5th decade and after) and may result in cardiac decompensation. Rarely, an ASD becomes manifest after myocardial infarction when changes in the compliance of the left ventricle result in a sudden increase in the intracardiac shunt, with consequent pulmonary edema and difficulty in oxygenation. Many patients with ASD are (or believe themselves) asymptomatic, but it is surprising how many asymptomatic people feel better after closure of the defect! When they are present, symptoms include fatigue, exertional breathlessness, and palpitations.

Right and Left Atrial Dissimilarities in Normal Subjects Explored by Speckle Tracking Echocardiography.

Atrial function is an important contributor of ventricular function and has a prognostic role in various cardiovascular diseases. We tested the hypothesis that right and left atrial (RA & LA) function may not be equal despite their accommodating identical cardiac output.

Double chambered right ventricle with ventricular septal defect in adults: case series and review of the literature.

Double-chambered right ventricle (DCRV) is an uncommon congenital anomaly in which anomalous muscle bands divide the right ventricle into two chambers; a proximal high-pressure and distal low-pressure chamber. It may be associated with mid right ventricular obstruction. It is commonly associated with other congenital anomalies, most frequently perimembranous ventricular septal defect (PM-VSD). We herein present 5 adult patients with concomitant DCRV and PM-VSD who varied in their symptomatic presentations and the ways of management.

Management of adults with congenital heart disease using videoconferencing across Western Canada: a 3-year experience.

BACKGROUND Management of adults with congenital heart disease (ACHD) requires quaternary centres with cardiologists and cardiac surgeons who have expertise in the diagnosis and management of this patient population. We report on the feasibility of the management of ACHD patients using videoconferencing and streaming through 1 regional referral centre covering 4 western Canadian provinces, roughly 30% of Canadas land and population. METHODS Videoconferencing sessions from January 2008 to December 2010 were systematically reviewed. Case presentations were classified as successful or unsuccessful. All patients were followed to assess whether the recommendations were acted upon. The hosting institution used the Alberta Health Services internet protocol network, while other connections used integrated service digital network. The videoconference equipment at the different sites includes Polycom HDX 9000 (Polycom, San Jose, CA), Tandberg Edge 95 (Tandberg, San Jose, CA), and Tandberg 990 (Tandberg). RESULTS From January 2008 to December 2010 there were 26 sessions, 213 case presentations, and 177 patients discussed with an average 8.2 case presentations per session. Thirty-two case presentations were deferred, 10 of which were because of transmission errors and the remainder were because of unavailability of staff or images. Of the 177 recommendations, 124 procedures (91 surgical, 29 percutaneous, and 4 electrophysiological) were booked directly at the regional referral centre. Only 6 recommendations were not carried out (4 because of premature deaths, and 2 because of patient logistic issues). CONCLUSIONS The results of this study illustrate that telehealth is a feasible medium for arriving at consensus recommendation in the management of ACHD patients living in a geographically diverse area.

Unusual Cardiac Infiltration in Diffuse Large B-Cell Lymphoma

A 38-year-old female presented with fever, shortness of breath and loss of appetite. Physical examination and electrocardiogram were unremarkable. Chest X-ray revealed pleural effusion and wide mediastinum. Contrast-enhanced computed tomography (CT) showed a large heterogeneous anterior mediastinal mass invading the mediastinal structures and left anterior chest wall with encirclement and compression of the main and left pulmonary arteries. The mass invaded the pericardium and was inseparable from the ventricular walls (Fig. 1A, B, and C). CT guided biopsy was obtained and histopathology was consistent with a high-grade diffuse large B-cell lymphoma. Fig. 1 Contrast-enhanced computed tomography showing a large heterogeneous anterior mediastinal mass (arrows) invading the mediastinal structures and left anterior chest wall with encirclement and compression of the main and left pulmonary arteries (A). The ... The patient underwent transthoracic echocardiogram (TTE) as part of routine work-up before chemotherapy. It uncovered normal left ventricular (LV) size with moderate systolic dysfunction and a large mass encircling the anterior and lateral LV walls causing akinesis (Fig. 1D, Supplementary movie 1). Cardiac magnetic resonance (CMR) confirmed the findings of TTE. The mass was hyperintense on T2-weighted and isointense on T1-weighted sequences with no evidence of perfusion. Additionally there was a non-ischemic sub-epicardial to mid wall late gadolinium enhancement involving the anterior wall with extension to a small portion of the anterior septum/anterolateral walls (Fig. 2, Supplementary movie 2). Those findings are in keeping with infiltration of the myocardial wall by the mediastinal lymphoma rather than an external compression. Fig. 2 A: Axial T1-weighted imaging showing the mass inseparable from the left ventricular wall (arrows). B: Short-axis T2-weighted imaging showing myocardial edema in the anterior and lateral walls of the left ventricle (arrowheads) with high signal intensity ... She was treated with 3 cycles of combination chemotherapy (R-ESHAP; rituximab plus etoposide, cytarabine, cisplatinum, and methylprednisolone). Follow-up contrast-enhanced CT showed marginal improvement of the size and extent of the mediastinal mass. Unfortunately, the patient died after completion of chemotherapy and her mode of death was unwitnessed. Diffuse large B-cell lymphoma is an uncommon category of primary mediastinal B-cell lymphoma that originates in the thymus. It is more prevalent in women and young adults. It usually presents with systemic symptoms, shortness of breath, chest discomfort, and palpable lymph nodes. It rarely manifests as an intracardiac mass and likely remains silent and often diagnosed on autopsy. With ventricular invasion, patients usually manifest with ventricular dysfunction which carries a dismal prognosis as noted in our patient.1),2),3) The incorporation of multi-modality imaging is very important in the diagnosis and management of cardiac/extracardiac masses. Discrimination between cardiac infiltration by lymphoma and primary cardiac tumors is complicated. CMR and contrast enhanced CT are the most valuable tools in demarcating direct invasion by nearby mediastinal masses from primary cardiac tumors.4),5) In our case, CMR was very useful in depicting a direct infiltration of the myocardium by the mediastinal lymphoma rather than intracavitary involvement or merely an external compression by the mass.

Patent Ductus Arteriosus with Eisenmenger Syndrome

Herein we report a 21 year-old woman with a previously documented patent ductus arteriosus and Eisenmenger physiology. She presented with increasing cyanosis and exercise intolerance which could be explained by a new finding of right to left shunting through an interatrial communication. She was started on Bosentan therapy aiming to reduce the pulmonary pressure with consideration for heart-lung transplantation should any further deterioration occur.

Successful catheter-based occlusion of persistent left-sided superior vena cava draining into the left atrium.

A 46-year-old woman presented with progressive fatigue. Her past history was remarkable for partial atrio-ventricular septal defect repair at the age of 9 years. Physical examination was notable for obesity, positional desaturation (PaO2 = 92% standing vs. 88% supine) and Grade 2/6 apical pansystolic murmur. Transthoracic echocardiography showed an intact atrial septum and mild mitral regurgitation. Saline contrast injection from the left arm showed prompt filling of both atria with bubbles consistent with a significant extracardiac right-to-left shunt and suggesting communication at the caval level (see …

Severe form of tetralogy of fallot: late presentation.

A 25-year-old man was evaluated due to progressive exercise intolerance and desaturation in the setting of previously documented Tetralogy of Fallot (TOF). He was diagnosed with TOF shortly after birth but his family declined surgery. Three years earlier, he had undergone left sided modified Blalock-Taussig shunt as a palliative procedure due to progressive shortness of breath and desaturation (O2 saturation between 80-85%). Initially, in the first few months after the procedure, he noticed slight improvement of his symptoms. However, he developed progressive exercise intolerance and desaturation which mandated complete repair. Physical examination was remarkable for desaturation (O2 saturation between 85-90%), clubbing and systolic ejection murmur (grade II-III/VI) over the pulmonic area. Electrocardiogram showed right axis deviation and right ventricular (RV) hypertrophy. Chest X-ray revealed dilated right side of the heart. Transthoracic echocardiogram (TTE) was technically difficult still showed dilated hypertrophied RV with normal systolic function, malalignment ventricular septal defect (VSD) and overriding of the aorta (Supplementary movie 1 and 2). Transesophageal echocardiogram (TEE) was carried out for further delineation of the RV outflow tract (RVOT) which was hypoplastic with a long (~4 cm) segment of narrowing and significant infundibular narrowing (by hypertrophied muscle bundle). Peak gradient across the RVOT was approximately 35-40 mm Hg (Fig. 1, Supplementary movie 3 and 4). Cardiac magnetic resonance (CMR) was technically challenging due to patients non-compliance with breathing instructions, yet confirmed the findings of TTE/TEE. Additionally, CMR uncovered hypoplastic proximal main pulmonary artery (PA), dilated left PA and mild proximal right PA stenosis with hypoplasia throughout the course (Fig. 2, Supplementary movie 5). The patient underwent successful complete repair with RV to PA conduit, resection of the native RVOT muscle bundles, arterioplasty of the right PA and VSD pericardial patch closure. Surgery was uneventful. Fig. 1 A: Transesophageal echocardiogram (TEE) transgastric view showing dilated right ventricle with significant hypertrophy. B: TEE apical long axis view showing overriding of the ascending aorta with malalignment ventricular septal defect (arrow). C: TEE ... Fig. 2 A: Cardiac magnetic resonance (CMR) sagittal image showing overriding of the ascending aorta with malalignment ventricular septal defect (arrow). B: CMR sagittal image showing hypoplastic right ventricular outflow tract and proximal main pulmonary artery ... An amalgamated approach of echocardiogram and CMR offers a precise detailed assessment of the RV, RVOT, and PA, which is critical for decision making regarding surgical intervention in this unusual case of unrepaired TOF presenting late in adult life.1),2)