Paul R. Julsrud

MR imaging of arrhythmogenic right ventricular cardiomyopathy: Morphologic findings and interobserver reliability

Background: Magnetic resonance (MR) imaging is frequently used to diagnose arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). However, the reliability of various MR imaging features for diagnosing ARVC/D is unknown. The purpose of this study was to determine which morphologic MR imaging features have the greatest interobserver reliability for diagnosing ARVC/D. Methods: Forty-five sets of films of cardiac MR images were sent to 8 radiologists and 5 cardiologists with experience in this field. There were 7 cases of definite ARVC/D as defined by the Task Force criteria. Six cases were controls. The remaining 32 cases had MR imaging because of clinical suspicion of ARVC/D. Readers evaluated the images for the presence of (a) right ventricle (RV) enlargement, (b) RV abnormal morphology, (c) left ventricle enlargement, (d) presence of high T1 signal (fat) in the myocardium, and (e) location of high T1 signal (fat) on a Likert scale with formatted responses. Results: Readers indicated that the Task Force ARVC/D cases had significantly more (χ2 = 119.93, d.f. = 10, p < 0.0001) RV chamber size enlargement (58%) than either the suspected ARVC/D (12%) or no ARVC/D (14%) cases. When readers reported the RV chamber size as enlarged they were significantly more likely to report the case as ARVC/D present (χ2= 33.98, d.f. = 1, p < 0.0001). When readers reported the morphology as abnormal they were more likely to diagnose the case as ARVC/D present (χ2 = 78.4, d.f. = 1, p < 0.0001), and the Task Force ARVC/D (47%) cases received significantly more abnormal reports than either suspected ARVC/D (20%) or non-ARVC/D (15%) cases. There was no significant difference between patient groups in the reported presence of high signal intensity (fat) in the RV (χ2 = 0.9, d.f. = 2, p > 0.05). Conclusions: Reviewers found that the size and shape of abnormalities in the RV are key MR imaging discriminates of ARVD. Subsequent protocol development and multicenter trials need to address these parameters. Essential steps in improving accuracy and reducing variability include a standardized acquisition protocol and standardized analysis with dynamic cine review of regional RV function and quantification of RV and left ventricle volumes.

Surgical treatment of vascular rings: the Mayo Clinic experience.

From 1947 through 1992, 37 Mayo Clinic patients underwent operation for the relief of tracheoesophageal obstruction that resulted from vascular rings and related entities. Of the 37 patients, 18 had a double aortic arch, 11 had a right aortic arch with an aberrant left subclavian artery, 4 had a left aortic arch with an aberrant right subclavian artery, 2 had a pulmonary artery sling, 1 had a right aortic arch with mirror-image branching and a left ligamentum arteriosum, and 1 had a left aortic arch, a right descending aorta, and a right ductus arteriosus. Symptoms consisted of stridor, recurrent respiratory infections, and dysphagia. The anomaly was approached through a left thoracotomy in 31 patients, through a right thoracotomy in 4, and through a median sternotomy in 2. Only one early postoperative death (3%) and no late deaths occurred. At long-term follow-up (maximal duration, 45 years), three patients had residual symptomatic tracheomalacia, one of whom required right middle and lower lobectomy for recurrent pneumonia. Magnetic resonance imaging is the imaging technique of choice for accurate delineation of the vascular and tracheal anatomy. When patients are symptomatic, vascular ring should be repaired. The surgical risk is minimal, and the long-term results are excellent.

Pulmonary Blood Supply in Patients With Pulmonary Atresia and Ventricular Septal Defect

The heart and lung specimens in 31 cases of pulmonary value atresia and ventricular septal defect were studied at autopsy. Three types of natural arterial blood supply to the lungs were identified: 1) ductus arteriosus (patient or ligamentous) (12 cases); 2) major collateral arteries (20 cases); and 3) diffuse small pleural arterial plexus coexisting with either ductus arteriosus or major collateral arteries (17 cases). The ductus arteriosus and major collateral arteries did not coexist in the same lung in these cases. Confluent central pulmonary arteries were present in 22 (71%) of the 31 cases, involving 7 (58%) of the 12 cases of ductus arteriosus, 14 (70%) of the 20 cases with major collateral arteries and 1 case with an aorticopulmonary window. The pulmonary trunk (atretic or patent) was identifiable in 24 (77%) of the 31 cases. A lung or lungs that connected to a ductus (or ligamentum) had a complete and unifocal intrapulmonary arterial distribution (without arborization abnormalities). Major collateral blood supply was frequently multifocal and associated with arborization abnormalities. The size of the central pulmonary arteries was not related to the type of arterial blood source but seemed to be related to the amount of blood flow actually reaching the vessels, This study demonstrated a complex systemic arterial system supplying the lungs in these cases. The size, sources and relation among the ductus, the pulmonary artery confluence, the large and small collateral vessels and the intrapulmonary system are far more varied than has ever been reported previously. Careful and thorough premortem studies are crucial if surgical intervention is contemplated.

Imaging strategies for vascular rings

Thirty-nine patients have undergone operation for relief of tracheoesophageal compression resulting from vascular rings and related entities at the Mayo Clinic. Nineteen patients had a double aortic arch, 11 patients had a right aortic arch with an aberrant left subclavian artery, 5 patients had a left aortic arch with an aberrant right subclavian artery, 2 patients had a pulmonary artery sling, 1 patient had a right aortic arch with mirror-image branching and a left ligamentum arteriosum, and 1 patient had a left aortic arch, a right descending aorta, and a right ductus arteriosus. Diagnostic examinations included chest radiography, barium esophagography, angiography, and, more recently, transthoracic echocardiography, computed tomography, and magnetic resonance imaging. A comparison among the various diagnostic techniques used in 12 patients during the last 12 years showed that angiography (n = 7), magnetic resonance imaging (n = 5), and computed tomography (n = 3) were the most reliable, as they always accurately delineated the anatomy. However, in the 6 patients who underwent transthoracic echocardiography, 1 of whom was an older child and 2 of whom were adults, the vascular abnormality was described correctly only once; in the other 5 patients, the results were false-negative or the technique failed to visualize the relevant vascular structures sufficiently. Currently, magnetic resonance imaging is our imaging technique of choice for the delineation of the vascular and tracheal anatomy in patients suspected of having a vascular ring.

Congenital Partial and Complete Absence of the Pericardium

Between 1952 and 1991, 15 Mayo patients were found to have partial or complete absence of the pericardium at the time of a cardiovascular surgical procedure. One patient with complete absence of the left pericardium had symptoms possibly related to the pericardial abnormality. This 42-year-old man had severe insufficiency of the tricuspid valve attributable to chordal rupture of the anterior leaflet, possibly precipitated by complete displacement of the heart into the left pleural space. Excision of the ruptured chordae and plication of the anterior flail leaflet rendered a competent tricuspid valve. In two patients, a small defect in the pericardium was repaired. Three patients who underwent operation for complex congenital heart disease died: two early postoperatively and one late after a reoperation. In the other 12 patients, no early or late postoperative complications were encountered. Although rare and usually asymptomatic, complete and partial deficiency of the pericardium may lead to serious complications such as cardiac valvular insufficiency or incarceration of cardiac tissue.

Effect of pulsed progressive fluoroscopy on reduction of radiation dose in the cardiac catheterization laboratory

The increased application of therapeutic interventional cardiology procedures is associated with increased radiation exposure to physicians, patients and technical personnel. New advances in imaging techniques have the potential for reducing radiation exposure. A progressive scanning video system with a standard vascular phantom has been shown to decrease entrance radiation exposure. The effect of this system on reducing actual radiation exposure to physicians and technicians was assessed from 1984 through 1987. During this time, progressive fluoroscopy was added sequentially to all four adult catheterization laboratories; no changes in shielding procedures were made. During this time, the case load per physician increased by 63% and the number of percutaneous transluminal coronary angioplasty procedures (a high radiation procedure) increased by 244%. Despite these increases in both case load and higher radiation procedures, the average radiation exposure per physician declined by 37%. During the same time, the radiation exposure for technicians decreased by 35%. Pulsed progressive fluoroscopy is effective for reducing radiation exposure to catheterization laboratory physicians and technical staff.

Early and late results of the modified Fontan procedure for doubleinlet left ventricle: The Mayo Clinic experience☆

Between May 1974 and March 1989, 155 patients with double-inlet left ventricle had the Fontan procedure performed at the Mayo Clinic. Age at operation ranged from nearly 2 to 41 years (median 10). The operative mortality rate from 1974 through 1980 (39 patients) was 21%, but from 1981 through 1989 (116 patients) it was reduced to 9%. The 17 late deaths were secondary to reoperation (n = 8), progressive myocardial failure (n = 5), sudden arrhythmia (n = 3) and bleeding varices (n = 1). Neither operative nor late mortality rate was significantly related to age at operation. At follow-up of 6 months to 11 years (mean 4.9 years) in 111 patients, 88% were in good or excellent condition and 12% were in fair or poor condition. The Fontan operation can be performed with a mortality risk of <10% in properly selected patients with double-inlet left ventricle. Late results are encouraging when contrasted with the clinical course of patients before this operative approach was utilized.

Valsalva Sinus Aneurysms: Findings at CT and MR Imaging

Aneurysms of the Valsalva sinus (aortic sinus) can be congenital or acquired and are rare. They are more common among men than women and among Asians than other ethnic groups. Nonruptured aneurysms may be asymptomatic and incidentally discovered, or they may be symptomatic and manifest acutely with mass effect on adjacent cardiac structures. Ruptured Valsalva sinus aneurysms result in an aortocardiac shunt and may manifest as insidiously progressive congestive heart failure, severe acute chest pain with dyspnea, or, in extreme cases, cardiac arrest. Although both ruptured and nonruptured Valsalva sinus aneurysms may have potentially fatal complications, after treatment the prognosis is excellent. Thus, prompt and accurate diagnosis is critical. Most Valsalva sinus aneurysms are diagnosed on the basis of echocardiography, with or without angiography. However, both electrocardiographically gated computed tomography and magnetic resonance (MR) imaging can provide excellent anatomic depiction, and MR imaging can provide valuable functional information.

The Fontan Procedure for Pulmonary Atresia With Intact Ventricular Septum: Operative and Late Results

OBJECTIVES The goals of the study were to evaluate the operative and late mortality associated with the Fontan procedure in patients with pulmonary atresia and an intact ventricular septum and to obtain follow-up information on the current clinical status of surviving patients. BACKGROUND Between 1979 and October 1, 1995, 40 patients with the anomaly had a nonfenestrated Fontan procedure performed at the Mayo Clinic. Because there are no previously published reports involving a series of this size in which the Fontan approach was used for this condition, a review of patient outcomes was thought to be of value. METHODS The medical records of the 40 patients were reviewed retrospectively, and 34 were determined to be alive. The status of the survivors as of late 1995 was then ascertained by direct examination, questionnaire or telephone follow-up. RESULTS There were three operative deaths and three late deaths. The current ages of the 34 survivors ranged from 4 to 30 years (median 13). Thirty-three of the 34 survivors were thought to be in New York Heart Association functional class I or II, and all but three of these patients, of school age or older, were either full-time students or working full time. The three adults who were not employed thought they were capable of working but were not doing so because of socioeconomic reasons. More than half of the patients were not receiving cardiovascular medications. CONCLUSIONS These overall gratifying early and late results encourage continued application of this operation for appropriately selected patients with this complex congenital cardiovascular anomaly.

Cardiac Transplantation for End-Stage Congenital Heart Defects: The Mayo Clinic Experience

OBJECTIVE To review the outcome of cardiac transplantation undertaken in patients with congenital heart defects. MATERIAL AND METHODS Between November 1991 and March 1998 at our institution, cardiac transplantation was performed in 16 patients with congenital heart disease (age range, 3 to 57 years; mean, 26.1). Preoperative diagnoses included univentricular heart (N = 4); complete transposition of the great arteries (N = 3); Ebsteins anomaly (N = 2); tetralogy of Fallot (N = 2); levotransposition (N = 2); dextrocardia, corrected transposition, ventricular and atrial septal defects, and pulmonary stenosis (N = 1); double-outlet right ventricle (N = 1); and hypertrophic obstructive cardiomyopathy (N = 1). All patients had undergone from one to five previous palliative operations. RESULTS Four patients required permanent pacemaker implantation during the first month postoperatively because of bradycardia; more than 2 years later, another patient required a permanent pacemaker because of sick sinus syndrome. In addition, one patient had an automatic implantable cardioverter-defibrillator. Three patients required reconstruction of cardiovascular structures with use of prosthetic material (Teflon patches or donor tissue) at the time of cardiac transplantation. Actuarial 1-, 2-, and 5-year survival was 86.2 +/- 9.1%. During the first year after transplantation, two deaths occurred--one at 41 days of putative vascular rejection and the second at 60 days of severe cellular rejection. All other patients are alive and functionally rehabilitated; the mean follow-up period has been 26.1 months (range, 2 to 89.6). CONCLUSION Cardiac transplantation for patients with congenital heart disease can be accomplished with a low perioperative mortality and an excellent medium-term survival despite the challenges presented by the technical difficulties during invasive diagnostic procedures and at operation and the need for adherence to long-term multiple-drug therapy in this patient population.