Frank Cetta

Nongeometric Quantitative Assessment of Right and Left Ventricular Function: Myocardial Performance Index in Normal Children and Patients with Ebstein Anomaly

Assessment of ventricular systolic function has been based on the geometric models of ventricular shape. This study was designed to define normal values for a nongeometric myocardial performance index (MPI) in children and to evaluate the utility of MPI in congenital heart disease. The MPI measures the ratio of total time spent in isovolumic activity (isovolumic contraction time and isovolumic relaxation time) to the ejection time. The right ventricular (RV) and left ventricular (LV) MPI were measured in 152 normal children (ages 3 to 18 years) and 45 preoperative patients with Ebstein anomaly (age 1 week to 52 years). In normal children, the RV MPI was 0.32+/-0.03 and the LV MPI was 0.35+/-0.03. In the Ebstein group, both RV and LV MPI were abnormally increased compared with age-matched normal subjects (Ebstein group: RV MPI=0.49+/-0.12, LV MPI=0.42+/-0.09, P < .001). Increasing RV dysfunction was associated with progressively increasing (abnormal) values of RV MPI (P < .001). The myocardial performance index quantitatively reflects ventricular performance in patients with complex ventricular geometry (ie, Ebstein anomaly). In the absence of a geometric solution, this nongeometric index is particularly appealing for the assessment of RV or LV performance.

Autoregulation of Cerebral Blood Flow in Fetuses with Congenital Heart Disease: The Brain Sparing Effect

Fetuses with congenital heart disease (CHD) have circulatory abnormalities that may compromise cerebral oxygen delivery. We believe that some CHD fetuses with decreased cerebral oxygen supply have autoregulation of blood flow that enhances cerebral perfusion (brain sparing). We hypothesize that cerebral autoregulation occurs in CHD fetuses, and the degree of autoregulation is dependent on the specific CHD and correlates with intrauterine head circumferences. CHD fetuses were compared to normal fetuses. Data included cardiac diagnosis, cerebral and umbilical artery Doppler, head circumference, weight, and gestational age. The cerebral-to-placental resistance ratio (CPR) was assessed as a measure of cerebral autoregulation. CPR = cerebral/umbilical resistance index (RI) and RI = systolic–diastolic/systolic velocity (normal CPR>1). CPR>1 was found in 95% of normal vs 44% of CHD fetuses. The incidence of CPR<1 was greatest in hypoplastic left or right heart fetuses. Compared to normal, cerebral RI was decreased in CHD fetuses. The CPR vs gestational age relationship, and the relationship among weight, head circumference, and CPR differed across normal and CHD fetuses. Fetuses > 2 kg with CHD and a CPR<1 had smaller head circumferences than normal. Brain sparing occurs in CHD fetuses. Fetuses with single ventricular physiology are most affected. Inadequate cerebral flow in CHD fetuses, despite autoregulation, may alter brain growth.

Improved early morbidity and mortality after Fontan operation: The Mayo Clinic experience, 1987 to 1992

OBJECTIVES This study sought to evaluate changes in early morbidity and mortality as well as predictors of outcome in our most recent 339 patients undergoing modified Fontan operations. BACKGROUND The Fontan operation is the preferred definitive palliation for patients with functional single ventricles. Previously reported early mortality rates after Fontan operation have been substantial. METHODS Records of 339 consecutive patients who had a Fontan operation at the Mayo Clinic between 1987 and 1992 (recent cohort) were reviewed. This cohort was compared with the previous 500 patients who had Fontan operations performed between 1973 and 1986 (early cohort). RESULTS Recently, overall early mortality after Fontan has decreased significantly compared with that for the early cohort (from 16% to 9%, p = 0.002). This decline occurred despite increased anatomic complexity of patients. Short-term posthospital survival has also improved significantly in recent patients. One-year survival improved to 88% from 79%, and 5-year survival to 81% from 73% (p = 0.006). Patients with common atrioventricular valves and those who took daily preoperative diuretic medication or had either postoperative renal failure or elevated postbypass right atrial pressure were at increased risk for early mortality. Young age was not found to be a risk factor for early mortality. Early mortality for patients with heterotaxia decreased dramatically: recent 30-day mortality was 15% compared with 41% in the early heterotaxy cohort. CONCLUSIONS Many factors may have contributed to decreased early mortality after Fontan. Improved patient selection, younger age at time of operation, refinements in surgical techniques and postoperative management may all have had important roles. Proposed technical modifications of the Fontan operation must be evaluated in light of these improved results.

Predictors of Early- and Late-Onset Supraventricular Tachyarrhythmias After Fontan Operation

BACKGROUND The objectives of our study were to determine the frequency of supraventricular tachyarrhythmias (SVTAs) among modifications of the Fontan operation and identify risk factors for developing SVTA. METHODS AND RESULTS The population consisted of all patients who had any modification of the Fontan operation at the Mayo Clinic between 1985 and 1993. Clinically significant SVTAs were those requiring initiation or change of antiarrhythmic treatment, and they were divided into early SVTAs (<30 days after the operation) and late SVTAs (>/=30 days after the operation). Clinical histories were reviewed, and health status questionnaires were sent. Four hundred ninety-nine patients had various modifications of the Fontan operation. Frequency of early SVTA was 15%. Risk factors identified by multivariate analysis for early SVTA were AV valve regurgitation, abnormal AV valve, and preoperative SVTA. Frequency of late SVTA was 6% by 1 year, 12% by 3 years, and 17% by 5 years. Risk factors for late SVTA were age at operation (<3 or >/=10 years) and systemic AV valve replacement. By univariate and multivariate analysis, the type of Fontan operation was not a significant risk factor for late SVTA when all 6 modifications were considered. However, when we analyzed the frequency of late SVTA for the 2 recently used modifications, we found a lower frequency of late SVTA in patients with atriopulmonary connection with lateral tunnel compared with those with total cavopulmonary connection. CONCLUSIONS Postoperative SVTA continues to be a significant problem. Risk factors for SVTA are AV valve regurgitation, abnormal AV valve, preoperative SVTA, and age at operation. Frequency of SVTA does not appear to be related to type of Fontan procedure except for slightly lower frequency in patients with atriopulmonary connection with lateral tunnel compared with those with total cavopulmonary connection.

40-Year Follow-Up After the Fontan Operation: Long-Term Outcomes of 1,052 Patients.

BACKGROUND There are limited long-term, single-cohort, follow-up studies available about patients after the Fontan operation. OBJECTIVES This study sought to determine the long-term outcome of all patients who had a Fontan operation at the Mayo Clinic. METHODS Records of all patients who had a modified Fontan operation between 1973 and 2012 were reviewed. A follow-up questionnaire was mailed to all patients alive at the time of the study. RESULTS Overall, 10-, 20-, and 30-year survival for 1,052 patients was 74%, 61%, and 43%, respectively. Factors associated with decreased overall or late survival in multivariate analysis included pre-operative diuretic use, longer cardiopulmonary bypass time, operation prior to 1991, atrioventricular valve (AVV) replacement at the time of Fontan operation, elevated post-bypass Fontan (>20 mm Hg) or left atrial (>13 mm Hg) pressures, prolonged chest tube drainage (>21 days), post-operative ventricular arrhythmias, renal insufficiency, and development of protein-losing enteropathy (PLE). Pre-operative and intraoperative sinus rhythm were associated with improved survival. Long-term survival was similar for patients regardless of ventricular morphology. The most common reoperations were pacemaker insertion/revision in 212 patients (20%), Fontan revision/conversion in 117 patients (11%), and AVV repair/replacement in 66 patients (5%). Clinically significant late atrial or ventricular arrhythmias occurred in 468 patients (44%). Ninety-five patients (9%) developed PLE, and 5-, 10-, and 20-year survival after diagnosis of PLE was 50%, 35%, and 19%, respectively. CONCLUSIONS As the surgical techniques for the Fontan operation have changed over the last 40 years, survival has improved. However, development of PLE and arrhythmias and the need for reoperation during long-term follow-up pose significant management challenges.

Quantitative Assessment of Fetal Ventricular Function: Establishing Normal Values of the Myocardial Performance Index in the Fetus

Objective: Assessment of ventricular function in the fetus has been limited for many reasons, including relative cardiac size and atypical orientation of fetal cardiac structures. A myocardial performance index (MPI) has been described in adult and pediatric populations as an echocardiographic measure of global (systolic and diastolic) ventricular performance. Because the MPI is a Doppler index, it is independent of ventricular geometry and can be applied to both left and right ventricular function. This study attempts to define the MPI in a group of normal fetuses and compare these data to other published studies of this index. Study Design: The right ventricular (RV) and left ventricular (LV) MPI were measured in 125 normal fetuses (20–40 weeks gestation, mean age 28 weeks). These fetuses were divided into five gestational age groups for comparison. These data were compared to 152 normal children (age 3–18 years, mean age 9.3 years). Results: In normal fetuses, the LV MPI was 0.36 ± 0.06 and the RV MPI was 0.35 ± 0.05. This was not statistically different from the group of normal children in whom the LV MPI was 0.35 ± 0.03 and the RV MPI was 0.32 ± 0.03. In addition, no significant change in the fetal MPI was seen with advancing gestational age. Conclusion: This study demonstrates that fetal ventricular function can be quantitatively measured with the MPI. The MPI provides an easily obtainable and reproducible measure of fetal ventricular performance that can be readily incorporated into all fetal echocardiographic examinations.

Clinical Outcomes and Improved Survival in Patients With Protein-Losing Enteropathy After the Fontan Operation

BACKGROUND Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis. OBJECTIVES The aim of this study was to review outcomes in patients with PLE following the Fontan operation. METHODS From 1992 to 2010, 42 patients (55% male) with PLE following the Fontan operation were identified from clinical databases at the Mayo Clinic. Data were collected retrospectively. RESULTS Mean age at PLE diagnosis was 18.9 ± 11.0 years. Initial Fontan operation was performed at 10.1 ± 10.8 years of age. Mean time from Fontan operation to PLE diagnosis was 8.4 ± 14.2 years. Survival was 88% at 5 years. Decreased survival was seen in patients with high Fontan pressure (mean >15 mm Hg; p = 0.04), decreased ventricular function (ejection fraction <55%; p = 0.03), and New York Heart Association functional class >2 at diagnosis (p = 0.04). Patients who died had higher pulmonary vascular resistance (3.8 ± 1.6 Wood units [WU] vs. 2.1 ± 1.1 WU; p = 0.017), lower cardiac index (1.6 ± 0.4 l/min/m(2) vs. 2.7 ± 0.7 l/min/m(2); p < 0.0001), and lower mixed venous saturation (53% vs. 66%; p = 0.01), compared with survivors. Factors were assessed at the time of PLE diagnosis. Treatments used more frequently in survivors with PLE included spironolactone (21 [68%]), octreotide (7 [21%]), sildenafil (6 [19%]), fenestration creation (15 [48%]), and relief of Fontan obstruction (7 [23%]). CONCLUSIONS PLE remains difficult to treat; however, in the current era, survival has improved with advances in treatment. Further study is needed to better understand the mechanism of disease and ideal treatment strategy.

Successful percutaneous repair of perivalvular prosthetic regurgitation

To examine the feasibility and outcome of percutaneous transcatheter repair of perivalvular regurgitation.

Usefulness of the myocardial performance index for early detection of Anthracycline-induced cardiotoxicity in children

Serial surveillance for cardiotoxicity in patients receiving anthracyclines has most commonly centered on the assessment of left ventricular (LV) systolic function by either shortening fraction or ejection fraction. A myocardial performance index (MPI) has been described as a noninvasive Doppler measurement of global (systolic and diastolic) ventricular function.1,2 The MPI has been shown to correlate well with other invasive and noninvasive measures of LV function in adults.3 Recent studies in pediatric and adult patients have demonstrated that this index can quantitatively assess right ventricular dysfunction as well. 4‐6 The MPI has also been shown to have significant clinical utility. Studies have demonstrated the MPI to be a powerful predictor of outcome in adult patients with dilated cardiomyopathy and primary pulmonary hypertension.7,8 This study evaluates the clinical utility of the MPI in the serial measurement of global left and right ventricular function in pediatric cancer patients who received anthracycline antibiotics. ••• The study population consisted of 26 pediatric patients who were treated with anthracycline antibiotics as part of their chemotherapeutic regimen for childhood malignancy (age range 0.5 to 18 years, mean 9.3 6 5.4). A group of normal children served as the control population; their characteristics have

Idiopathic Restrictive Cardiomyopathy in Childhood: Diagnostic Features and Clinical Course

OBJECTIVE To describe the clinical course and outcome of children with idiopathic restrictive cardiomyopathy (IRCM) and to present the Doppler echocardiographic features of this disease in childhood. DESIGN We reviewed the Mayo Clinic patient database for the period from 1975 to 1993 to identify children who underwent assessment for IRCM. MATERIAL AND METHODS Clinical records and diagnostic studies, including two-dimensional (2-D), M-mode, and Doppler echocardiograms, were reviewed for each patient. Characteristics were analyzed statistically to determine potential predictors of outcome. RESULTS Eight children (five girls and three boys) were diagnosed with IRCM between 1975 and 1993 at our institution. The median age at diagnosis was 11 years, and the median duration of follow-up was 11.5 years. Of the eight patients, five died (the median time from initial examination to death was 1 year). All five of these patients had clinical and radiographic evidence of pulmonary venous congestion. In all patients, 2-D and M-mode echocardiography revealed atrial enlargement without ventricular dilatation or hypertrophy. The four patients who underwent detailed diastolic Doppler assessment had findings consistent with restrictive filling and increased left ventricular end-diastolic pressure: (1) short mitral deceleration time, (2) increased pulmonary vein atrial reversal velocity and duration, and (3) pulmonary vein atrial reversal duration greater than mitral A-wave duration. CONCLUSION The prognosis for children with IRCM is poor. In this small group of patients, absence of pulmonary venous congestion most consistently predicted extended survival. A combined 2-D and Doppler echocardiographic examination provides a reliable noninvasive means of assessing the physiologic and morphologic features of IRCM in children.