Michael S. Connelly

Arrhythmia and Mortality After the Mustard Procedure: A 30-Year Single-Center Experience

OBJECTIVES Our purpose was to assess the risk factors for late mortality, loss of sinus rhythm and atrial flutter after the Mustard operation. BACKGROUND The Mustard operation provides correction of cyanosis with low surgical risk in transposition of the great vessels. However, right ventricular failure, loss of sinus rhythm, atrial flutter and death are frequent long-term complications. METHODS Records of 534 children who underwent the Mustard operation at a single center since 1962 were reviewed for demographic, anatomic, electrocardiographic and physiologic predictors and outcomes. RESULTS There were 52 early deaths (9.7%). Survival analysis was undertaken for 478 early survivors with a mean follow-up interval of 11.6 +/- 7.2 years. There were 77 late deaths (16.1%), with sudden death (n = 31) the most frequent cause. Survival estimates were 89% at 5 years and 76% at 20 years of age. Risk factors were an earlier date of operation, operative period arrhythmia and an associated ventricular septal defect. Risk (hazard) of late death declined in the first decade, with further peaks in the second decade. Sinus rhythm was present in 77% at 5 years and 40% at 20 years. Loss of sinus rhythm was associated with previous septectomy, postoperative bradycardia and late atrial flutter. Freedom from atrial flutter was 92% at 5 years and 73% at 20 years of age. Risk factors for atrial flutter were the occurrence of perioperative bradyarrhythmia, reoperation and loss of sinus rhythm during follow-up. Risk of atrial flutter demonstrates a late increase. CONCLUSIONS Ongoing loss of sinus rhythm and late peaks in the risk of atrial flutter and death necessitate continued follow-up.

Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: Outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan’s syndrome

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part II of the guidelines includes recommendations for the care of patients with left ventricular outflow tract obstruction and bicuspid aortic valve disease, coarctation of the aorta, right ventricular outflow tract obstruction, tetralogy of Fallot, Ebstein anomaly and Marfans syndrome. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

Effect of angiotensin receptor blockade on systemic right ventricular function and size: A small, randomized, placebo-controlled study

INTRODUCTION The effects of angiotensin converting enzyme inhibitor on systemic right ventricular size and function are unknown. METHODS Prospective, double blind, randomized, placebo-controlled clinical trial of 1-year therapy with Ramipril in adult patients with DTGA status postatrial baffle procedure. Primary endpoints were change in systemic right ventricular ejection fraction (RVEF) and right ventricular size assessed by magnetic resonance imaging (MRI). RESULTS Seventeen patients were enrolled into the study. Mean age at study enrollment was 26.4+/-5.2 years. Mean baseline RVEF was 44+/-6.5%, mean RVEDV was 206.3+/-75.5 ml. Eight patients were randomized to the treatment group and 9 patients were randomized to the placebo group. RVEF did not improve in the Ramipril group from baseline to 1 year (43.8+/-7.1% vs. 40.9+/-13.3%, p=0.52) and remain unchanged in the placebo group (44.3+/-6.3 vs. 46.3+/-9.6%, p=0.42). RVEDV (184.5+/-56.4 ml vs. 179.6+/-66.4 ml, p=0.64) and RVESV (109.5+/-19.4 ml vs. 111.8+/-30.1, p=0.74) remained unchanged in the Ramipril group from baseline to 1 year as well as in the placebo group (228.1+/-89.2 ml vs. 204.5+/-50.4 ml, p=0.42 and 117.5+/-36.9 ml vs. 117.4+/-26.2 ml, p=0.99, respectively). CONCLUSION One-year treatment with Ramipril does not seem to affect right ventricular function or size in adult patients with systemic right ventricles after a Mustard or Senning procedure. CONDENSED ABSTRACT 17 Adult patients (mean age of 26.4 +/- 5.2 years) with systemic right ventricles were blindly randomized to 1-year treatment with Ramipril placebo. Systemic right ventricular function (RVEF) and size (RVEDV) were assessed by magnetic resonance imaging at baseline and 1 year. RVEF failed to improve in the Ramipril group (43.8+/-7.1% vs. 40.9+/-13.3%, p=0.52) and remain unchanged in the placebo group (44.3+/-6.3 vs. 46.3+/-9.6%, p=0.42). RVEDV remained unchanged in the study group (184.5+/-56.4 ml vs. 179.6+/-66.4 ml, p=0.64 as well as in the placebo group (228.1 +/- 89.2 ml vs. 204.5 +/- 50.4 ml, p = 0.42). One-year treatment with Ramipril does not improve right ventricular function or attenuate remodeling in adult patients with systemic right ventricles.

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.

Unforeseen cardiac involvement in McArdle's disease.

McArdles disease (glycogen storage disease type V) is a rare autosomal recessive metabolic myopathy due to myophosphorylase deficiency. It classically manifests by exercise intolerance, leg cramps, muscle pain and occasionally exercise induced myoglobinuria. The onset of exercise intolerance is typically in the second or third decades of life. It has a specific predilection to skeletal muscle involvement, yet cardiac muscle involvement is very rare. This report describes an unusual case of a 33 year-old man with known McArdles disease who presented with an incidental finding of severe obstructive hypertrophic cardiomyopathy.

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.

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.

Reticent Uneventful Rupture of Right Coronary Sinus of Valsalva Aneurysm into Right Ventricle

Aneurysms of sinus of Valsalva are rare congenital or acquired cardiac anomalies. They most commonly involve the right or non-coronary sinuses. Herein we report a very rare case of a ruptured congenital right sinus of Valsalva aneurysm into the right ventricular outflow tract followed conservatively for at least 10 years without any events.

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.