Patrick W. O'Leary

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.

Diastolic Ventricular Function in Children: A Doppler Echocardiographic Study Establishing Normal Values and Predictors of Increased Ventricular End-Diastolic Pressure

OBJECTIVE To extend noninvasive assessment of diastolic cardiac function into the pediatric age-group. DESIGN This study was divided into two phases, the first of which was designed to provide an age-appropriate set of normal diastolic Doppler echocardiographic data for children and adolescents and the second of which was to determine whether these Doppler techniques could be used to identify children with increased ventricular end-diastolic pressure (EDP). MATERIAL AND METHODS Complete echocardiographic studies focusing on Doppler variables of diastolic ventricular function were performed on 223 normal children. Values observed were analyzed for dependence on age, heart rate, and gender. Results from the normal group were then compared with Doppler values observed in a group of 24 children with catheterization-substantiated increases in ventricular EDP. RESULTS Normal values for the Doppler factors studied vary with both age and heart rate. The variables that most confidently distinguished children with increased EDP from normal subjects were the ratio of and the difference between the durations of pulmonary vein atrial reversal and the mitral A wave. A ratio of 1.2 or more or a difference of 29 ms or more identified those children with increased EDP with sensitivities of 88 and 90% and specificities of 86 and 86%, respectively. CONCLUSION Use of the normal data and the Doppler techniques described in this study will allow confident assessment of diastolic function in children as well as in adults.

Left Heart Lesions in Patients With Ebstein Anomaly

OBJECTIVE To identify the incidence of left heart abnormalities in patients with Ebstein anomaly, recognizing that left-sided lesions in this patient group have been overlooked. PATIENTS AND METHODS According to the echocardiography database at the Mayo Clinic in Rochester, Minn, 106 consecutive patients with Ebstein anomaly underwent echocardiography between July 1, 2001, and February 28, 2003. Clinical data as well as electrocardiographic and echocardiographic reports and images were reviewed. RESULTS Ebstein anomaly was severe in 76 patients (72%). Previous tricuspid valve surgery was reported in 46 patients (43%), and previous closure of an atrial septal defect or patent foramen ovale was reported in 34 patients (32%). Left ventricular (LV) myocardial changes resembling noncompaction occurred in 19 patients (17.9%), LV systolic dysfunction in 7 patients (7%), LV diastolic dysfunction in 34 (36%) of 95 patients, and LV dilatation in 4 patients (4%). Additional left-sided cardiac lesions included mitral valve prolapse in 16 patients (15%), bicuspid aortic valve in 8 (8%), mitral valve dysplasia in 4 (4%), and ventricular septal defect in 8 (8%). Wolff-Parkinson-White syndrome occurred in 22 patients (21%). The QRS axis tended to be different in LV noncompaction with a mean +/- SD axis of 12 degrees +/- 74 degrees vs 36 degrees +/- 66 degrees overall (P=.08). Otherwise, there were no differences in clinical or surgical data between the groups with normal and abnormal LV myocardium. CONCLUSIONS In patients with Ebstein anomaly, left heart abnormalities involving the myocardium or valves were observed in 39% of patients. Ebstein anomaly should not be regarded as a disease confined to the right side of the heart.

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.

Anatomic repair of Ebstein's malformation: lessons learned with cone reconstruction.

BACKGROUND Reproducible repair of Ebsteins malformation is challenging and numerous surgical techniques have been described. We reviewed our experience with the cone reconstruction. METHODS Between June 2007 and December 2011, 89 patients (47 female; 53%) underwent cone reconstruction (median age 19 years; range, 19 days to 68 years). Indication for operation was progressive cardiomegaly in 43 (48%), cyanosis in 29 (33%), and heart failure in 13 (15%). Prior tricuspid valve repair was performed in 12 patients (13%). Severe tricuspid regurgitation (TR) was present in 75 patients (84%). RESULTS All patients underwent cone reconstruction (360-degree leaflet tissue repair anchored at true annulus). Modifications included ringed annuloplasty in 57 patients (64%), leaflet augmentation in 28 patients (31%), and autologous chordae in 17 patients (19%). Bidirectional cavopulmonary anastomosis was performed in 21 patients (24%). Early mortality occurred in 1 patient (1%). Early reoperation for recurrent TR occurred in 12 patients (13%); re-repair was performed in 6 patients (50%), and 6 (50%) required replacement. Mean follow-up was 19.7 ± 24.7 months. There was no late mortality or reoperation. At follow-up, 72 patients (87%) had no or mild TR, 9 (11%) had moderate TR, and 2 patients (2%) had severe TR. Ringed annuloplasty was associated with less than moderate TR at dismissal (p = 0.01). CONCLUSIONS The learning curve for cone reconstruction is steep, but early mortality is low. Cone reconstruction with ringed annuloplasty results in less TR and should be used whenever possible. Longer follow-up is essential to determine late durability of cone reconstruction.

Surgical treatment of Ebstein's malformation: state of the art in 2006

Ebsteins malformation is a congenital anomaly of the tricuspid valve and right ventricle that is characterized by several features, each of which can exhibit a spectrum of malformation. The first is adherence of the leaflets of the tricuspid valve to the underlying myocardium, this representing failure of delamination during development. This feature involves the septal and inferior leaflets, but rarely the anterior leaflet ( Fig. 1 ). The second feature is anterior and apical rotational displacement of the functional annulus ( Fig. 2 ). The third abnormality is dilation of the “atrialized” portion of the right ventricle, with variable degrees of hypertrophy and thinning of the wall. The fourth finding is redundancy, fenestrations, and tethering of the anterior leaflet. A fifth abnormality is dilation of the right atrioventricular junction, this being the true tricuspid valvar annulus. The final feature is variable ventricular myocardial dysfunction. Each heart with Ebsteins malformation is different, and there is an infinite variability that can occur with the above mentioned characteristics. These anatomical and functional abnormalities cause important tricuspid regurgitation, which results in right atrial and right ventricular dilation, and atrial and ventricular arrhythmias.

Measurement, interpretation and use of haemodynamic parameters in pulmonary hypertension associated with congenital cardiac disease.

I N PULMONARY ARTERIAL HYPERTENSION, ASSESSMENT of pulmonary haemodynamics by means of cardiac catheterization is crucial to confirm diagnosis and establish the potential efficacy of vasodilator therapy. In the specific setting of pulmonary hypertension associated with congenital cardiac disease, the reasons for obtaining haemodynamic parameters go far beyond choosing and evaluating the effects of drugs, encompassing the need for defining operability and deciding between correction, palliation, and combination of surgical and medical treatments. The situation, therefore, is considerably different from assessment of those with acquired as opposed to congenital malformations. In terms of defining operability, the frequency with which cardiac catheterization is needed tends to decrease in developed countries. Surgical correction of anomalies associated with significant left-to-right shunting in early infancy avoids damage to the pulmonary circulation from increased blood flow and pressure. Some patients, nonetheless, present early in life with clinical features suggestive of markedly elevated pulmonary vascular resistance, hence representing a challenge for assessment by non-invasive methods alone. This is seen more frequently with certain anomalies such as transposed arterial trunks, common arterial trunk, and atrioventricular septal defects. Even in the setting of simple malformations, some infants and young children very early in life can display elevated pulmonary vascular resistance, in the absence of a history of pulmonary congestion or failure to thrive, with moderately sized or large defects. On the other hand, in developing countries, patients with large shunts frequently seek medical care after reaching 2 years of age. In these instances, definition of operability based on noninvasive evaluation is unrealistic. In the presence of shunts, the precise flows into the pulmonary and systemic circulations, and vascular resistances in these circuits, cannot be assessed by thermodilution, and are generally measured using the Fick principle. This involves a number of methodological problems and sources of errors. Furthermore, the pulmonary circulation needs to be challenged by vasodilators to provide a full understanding of the functional component of vascular resistance, a major determinant of operability. In small infants and children, the whole procedure is carried out under general anaesthesia, and anaesthetic drugs are known to induce undesirable changes in pulmonary and systemic haemodynamics. In these situations, appropriate selection of the anaesthetic protocol plays an important role in obtaining accurate haemodynamic data. We will discuss this issue in our section devoted specifically to anaesthetic protocols. Our overall aim, however, Correspondence to: Antonio Augusto Lopes, M.D., Director, Dept. Paediatric Cardiology and Adult Congenital Heart Disease, The Heart Institute (InCor) – HC.FMUSP, Av. Dr. Eneas de Carvalho Aguiar, 44, 05403-000 – São Paulo – Brazil. Tel: 55-11-3069-5350; Fax: 55-11-3069-5409; E-mail: aablopes@usp.br

Serial Doppler Assessment of Diastolic Function before and after the Fontan Operation

Serial Doppler echocardiography was used to evaluate the effects of the Fontan operation (FO) on diastolic ventricular function in 55 patients with univentricular heart. Examinations were performed before operation, before postoperative discharge, and 6 months to 6 years postoperatively. Preoperatively, early diastolic atrioventricular valve (E) flow was reduced and deceleration times prolonged relative to healthy children. All pulmonary vein diastolic flow variables, except diastolic velocity, were increased relative to control subjects. After FO, E/atrial velocity and E/atrial time velocity integral ratios were decreased whereas deceleration and isovolumic relaxation time remained constant. Diastolic pulmonary vein forward flow increased after FO. These data suggest inherently abnormal ventricular relaxation in the univentricular heart and that changes in flow patterns observed postoperatively were subtle and likely a result of reduced ventricular preload after FO. Overall, diastolic function most probably remained stable after FO. This information can be used as a benchmark for further diastolic function assessment in patients with surgically palliated univentricular heart.

Hand-carried ultrasound-guided pericardiocentesis and thoracentesis

To date, ultrasound-guided centeses have required the use of large, diagnostic ultrasound equipment that can be cumbersome and not readily available. In this study, we sought to evaluate the use of portable, hand-carried ultrasound devices in guidance of pericardiocentesis and thoracentesis. Hand-carried ultrasound was used to define location of the maximal extent of the effusion and its proximity to the skin surface, confirm needle entry and site, and to evaluate postprocedural result. A total of 12 patients who were stable and hospitalized were prospectively enrolled, 9 for pericardiocentesis and 3 for thoracentesis. We concluded that under the direction of a suitably trained physician, hand-carried ultrasound provided images suitable for guidance of a pericardiocentesis or thoracentesis preprocedurally, intraprocedurally, and postprocedurally.

Regenerative therapy for hypoplastic left heart syndrome: first report of intraoperative intramyocardial injection of autologous umbilical-cord blood-derived cells.

The current standard of care for neonates with hypoplastic left heart syndrome (HLHS) includes a series of cardiac operations culminating in a complete cavopulmonary connection. Given the increased workload placed on the right ventricle, development of ventricular dysfunction and heart failure are concerns. As this patient population ages, many may require cardiac transplantation. Cellbased strategies to treat heart failure in adults have been reported, but little is known about their feasibility and efficacy in children with congenital heart disease. In an effort to attenuate or prevent the progression of heart failure that leads to the need for cardiac transplantation, we have developed an autologous cell-based protocol for infants with HLHS. We present the first reported case of direct intramyocardial injection of umbilical-cord blood–derived mononuclear cells in an infant with HLHS undergoing stage-II surgical palliation.