Michele A. Frommelt

Prospective echocardiographic diagnosis and surgical repair of anomalous origin of a coronary artery from the opposite sinus with an interarterial course

OBJECTIVES In this study, we sought to describe the mode of presentation, anatomic features, diagnostic techniques, and surgical outcome in a group of patients with anomalous origin of a coronary artery from the opposite sinus with an interarterial course between the great arteries (AOCA). BACKGROUND Anomalous origin of a coronary artery from the opposite sinus with an interarterial course is associated with myocardial ischemia and sudden cardiac death, particularly in adolescents and young adults. METHODS The cardiology database at Childrens Hospital of Wisconsin was reviewed to identify all patients diagnosed with AOCA. RESULTS From September 1997 to August 2002, 10 patients were identified with AOCA; all were children/adolescents (age range, 3 months to 20 years; weight range, 4.7 to 72 kg), and nine were diagnosed prospectively by transthoracic echocardiography (TTE). Symptoms of cardiac ischemia initiated investigation in 4/10 patients at a mean age of 16 +/- 2.8 years; the other six had TTE for suspected congenital heart disease/musculoskeletal chest pain. The left coronary artery originated from the right sinus in six patients, and the right coronary artery originated from the left sinus in four patients. An intramural course of the AOCA within the anterior aortic wall was found in 9/10 patients and was reliably identified by TTE; the other patient had an intramyocardial course of the anomalous coronary. Surgical repair was performed in 8/10 patients at a mean age of 13 +/- 4.7 years. Unroofing of the intramural portion of the AOCA to relocate the ostia in the appropriate sinus was successfully performed in seven patients. All patients status post unroofing were asymptomatic with patent coronary flow by Doppler and normal exercise treadmill testing at a median follow-up interval of 1.5 years. CONCLUSIONS Anomalous origin of a coronary artery from the opposite sinus with an interarterial course is frequently characterized by an intramural course, which can be prospectively identified by TTE. Unroofing the intramural segment without bypass grafting can reliably repair the intramural form of AOCA.

Twenty-year experience with repair of complete atrioventricular septal defects

BACKGROUND To determine factors predicting mortality and morbidity after repair of complete atrioventricular septal defect, we retrospectively analyzed preoperative, operative, and postrepair factors on the outcome of 115 consecutive complete atrioventricular septal defect repairs at The Childrens Hospital of Wisconsin between January 1974 and December 1993. METHODS For the entire experience the operative mortality was 13.9% (16 patients). During the most recent era, January 1988 to December 1993, operative mortality was 3.6% (2 of 55 patients). This was significantly improved from the two previous eras, January 1974 to December 1980, 28% (7 of 25) and January 1981 to December 1987, 20% (7 of 35 patients) (p = 0.02). There were seven late deaths; 10-year actuarial survival, including operative mortality was 81%. Age at complete repair decreased; before 1982 all patients were more than 12 months of age, whereas after 1982 64% (56 of 88 patients) were 12 months of age or less. RESULTS Moderate or severe preoperative left atrioventricular valve regurgitation was not a risk factor for operative mortality. For operative survivors with moderate to severe preoperative left atrioventricular valve regurgitation (n = 17), late postoperative left atrioventricular valve regurgitation (follow-up data available on 15 patients) was significantly reduced (severe = 1, moderate = 5, mild = 9; p = 0.007). CONCLUSIONS Early mortality was predicted by the era of surgical repair. Conversion to routine repair during infancy was achieved with a simultaneous decrease in operative mortality. For patients with moderate to severe preoperative left atrioventricular valve regurgitation, significant improvement in the degree of left atrioventricular valve regurgitation can be expected without an increase in operative or late mortality or morbidity.

Ten-year experience with surgical unroofing of anomalous aortic origin of a coronary artery from the opposite sinus with an interarterial course

BACKGROUND Anomalous aortic origin of a coronary artery from the opposite sinus with an intramural course between the great arteries (AAOCA) is associated with ischemia and sudden cardiac death in children, and surgical unroofing has been used to alleviate that risk. METHODS The cardiology database was reviewed to identify all patients with AAOCA who underwent surgical unroofing 10 years. RESULTS From March 1999 to September 2009, 27 patients with a mean age of 12.6 ± 3.5 years (range, 4-20 years) had surgical unroofing of AAOCA of the left coronary from the right sinus (left AAOCA, 7/27, 26%) or of the right coronary from the left sinus (right AAOCA, 20/27, 74%). Of these, 26 had diagnoses made by transthoracic echocardiography. Symptoms included resuscitated sudden cardiac death in 3, syncope in 8, and chest pain in 4. No symptoms were present in 12 cases of serendipitous diagnosis. Unroofing of the intramural portion was successfully performed in all cases. A slitlike coronary orifice was described at surgical inspection in 12 patients, 7 of whom had right AAOCA and no symptoms. All patients after unroofing have patent coronary flow by Doppler and normal echocardiography and exercise treadmill testing at mean follow-up of 1.8 years. None have activity restrictions. CONCLUSIONS AAOCA is frequently characterized by an intramural course, which can be reliably identified by echocardiography. This form can be safely repaired by unroofing the intramural segment without early morbidity. Symptoms of possible ischemia are common but not always correlated with coronary ostial findings at surgery.

Usefulness of Doppler tissue imaging analysis of tricuspid annular motion for determination of right ventricular function in normal infants and children

We conclude that RV systolic and diastolic function can be assessed noninvasively using DTI analysis of tricuspid annular motion. This study describes tricuspid annular motion patterns in normal infants and children and should provide the foundation because this technique is applied to infants and children with altered RV anatomy and hemodynamics.

Psychological distress in parents of children with severe congenital heart disease : the impact of prenatal versus postnatal diagnosis

Objective:The purpose of the study was to evaluate coping and psychological functioning of parents of children prenatally or postnatally diagnosed with congenital heart disease.Study Design:Parents of 10 infants prenatally diagnosed by fetal echocardiogram and 7 infants postnatally diagnosed with severe congenital heart disease completed the Brief Symptom Inventory (BSI) at the time of diagnosis, at the time of birth and 6 months after birth. Prenatal and postnatal groups were compared to each other and to BSI norms.Result:Although both groups scored higher than test norms at the time of diagnosis, they did not differ significantly from each other. Six months after birth, the postnatal group scores did not differ from test norms, but the prenatal group scores were still significantly higher than test norms. The severity of the childs heart lesion at diagnosis was related to parental distress levels; parents with children with more severe lesions had higher BSI scores.Conclusion:Results suggest the need to provide parents with psychological support, regardless of the timing of diagnosis. Parents of children with more severe lesions may be at risk for higher levels of psychological distress, particularly over time.

Does an Additional Source of Pulmonary Blood Flow Alter Outcome After a Bidirectional Cavopulmonary Shunt

BACKGROUND The bidirectional cavopulmonary shunt has become an important intermediate step in the treatment of pediatric patients with single ventricle physiology who are ultimately destined for palliative surgery. We wanted to know whether there would be risks or benefits if an additional source of pulmonary blood flow was left after a bidirectional cavopulmonary shunt. METHODS AND RESULTS We retrospectively reviewed the medical and surgical records of all patients who underwent a bidirectional cavopulmonary shunt at the Childrens Hospital of Wisconsin between January 1991 and December 1993. A total of 43 patients were identified. Anatomic diagnoses included double inlet left ventricle (14 patients), tricuspid atresia (8 patients), pulmonary atresia with intact septum (6 patients), single right ventricle (5 patients), hypoplastic left heart (3 patients), unbalanced atrioventricular septal defect (3 patients), and other complex lesions (4 patients). We then divided the patients into two groups for purposes of analysis. Group 1 had only the cavopulmonary shunt as a source of pulmonary flow (22 patients); group 2 had an additional source of pulmonary flow (21 patients). Patient age at the time of cavopulmonary shunt ranged from 6 months to 12 years, with group 1 patients being younger (31 versus 45 months, P = .05). Group 2 patients had higher postoperative central venous pressures (17.8 versus 14.1 mm Hg, P < .001) and oxygen saturations (86% versus 81%, P < .001) than did group 1 patients. There was no statistical difference between groups in the number of chest tube days or hospital days. There was 1 early death in group 1 related to severe ventricular dysfunction and 1 late death in group 2 related to sepsis. Five patients in group 2 were readmitted to the hospital for drainage of a large chylothorax compared with none in group 1 (P < .02). CONCLUSIONS We conclude that patients with an additional source of pulmonary blood flow after bidirectional cavopulmonary shunt have higher postoperative central venous pressures, have higher oxygen saturations, and are at risk for the late development of a chylothorax.

Echocardiographic assessment of subvalvular aortic stenosis before and after operation

The development of two-dimensional and Doppler echocardiography has provided a noninvasive technique for the diagnosis and serial assessment of patients with subvalvular aortic stenosis. The clinical records and echocardiographic data were reviewed of all patients with subaortic stenosis diagnosed between 1983 and 1991. Of the 77 patients identified (45 male and 32 female), 28 had isolated subaortic stenosis and 49 had associated cardiac lesions. The most frequently encountered associated lesions were ventricular septal defect (n = 19) and coarctation of the aorta/interrupted aortic arch (n = 14). Serial echocardiographic studies, performed in 38 of the 77 patients, documented significant progression of the left ventricular outflow tract gradient in 25 patients (66%) and development of aortic regurgitation in 25 patients (66%). Surgical resection was performed in 36 patients. The preoperative outflow tract peak gradient was 62.9 +/- 31 mm Hg (range 0 to 153), whereas the immediate postoperative gradient was 14.4 +/- 14 mm Hg (range 0 to 67). The two patients with a significant residual gradient (37 and 67 mm Hg, respectively) in the immediate postoperative period had severe subaortic stenosis preoperatively with marked left ventricular hypertrophy and intracavitary gradient. The immediate postoperative echocardiograms demonstrated no worsening of aortic regurgitation in any patient and regression of regurgitation in one patient from mild to none. Intermediate-term follow-up studies were available for review in 13 postoperative patients at a mean of 4 years postoperatively. In 2(15%) of these 13 patients, subaortic stenosis recurred; however, the degree of aortic regurgitation did not increase in any patient.(ABSTRACT TRUNCATED AT 250 WORDS)

Perioperative monitoring in high-risk infants after stage 1 palliation of univentricular congenital heart disease

OBJECTIVE Survival of high-risk patients with univentricular heart disease after Norwood palliation is reduced. We hypothesized that early goal-directed monitoring with venous oximetry and near-infrared spectroscopy would offset their increased vulnerability and improve survival. METHODS A prospective database of patients undergoing stage 1 palliation was used to assess differences in outcomes across risk groups in the setting of a comprehensive, goal-directed monitoring program. High-risk criteria included gestational age 35 weeks or less, birth weight less than 2.5 kg, and additional cardiac or extracardiac anomalies. Outcomes included survival to defined end points and measures of postoperative support. RESULTS From September 2000 to September 2008, 162 patients underwent stage 1 palliation: 28% (45/162) high-risk and 72% (117/162) standard-risk patients. Lesions other than hypoplastic left heart syndrome were more common among high-risk patients (38%, 17/45, vs 15%, 18/117, P = .003). Operative survival was not statistically different(87%, 39/45, high risk vs 95%, 111/117, standard risk, P = .1). High-risk patients were more likely to receive inpatient treatment until stage 2 palliation (24%, 11/45, vs 10%, 12/117, P = .001) and had lower 1-year survival (78% vs 93%, P = .01) and survival to date (71% vs 92%, P = .001). CONCLUSIONS Intensive monitoring partially offset biologic vulnerability of high-risk patients, helping attain comparable early outcomes. Vulnerability persisted throughout the interstage period, however, and increased mortality beyond cavopulmonary shunt was seen only among high-risk patients. Although enhanced monitoring reduced early mortality, high resource use and attrition after stage 2 palliation suggest an ongoing need to evaluate our current palliative strategy for this subset of patients.

The impact of abnormal fetal echocardiography on expectant parents’ experience of pregnancy: a pilot study

To examine the impact of an abnormal fetal echocardiogram (echo) on parental attitudes, emotions and coping strategies. Group interviews were performed for women and their male partners who had a fetal echo and were found to have a fetal congenital heart defect. The women and men were separated into four different focus groups. The interviews were tape recorded, transcribed and analyzed. The women expressed strong emotions of guilt, fear and sadness or hopelessness. They coped by attaching and bonding to the fetus, and were realistic about the future. The women began grieving at the time of the fetal echo. The men experienced emotions of anger and anxiety. They coped by remaining optimistic, and focused on supporting their wives. Men grieved after the birth of the baby. The fetal echo was felt to be beneficial and not harmful by the parents. The fetal echo led to significant changes in the couples’ perceptions of themselves and their relationships. Clinicians performing fetal imaging must be aware of parental affective responses and coping mechanisms in order to provide the required psychological support.

Aprotinin improves outcome of single-ventricle palliation

BACKGROUND Elevation of pulmonary vascular resistance as a consequence of cardiopulmonary bypass may lead to failure of single-ventricle palliation. We reviewed our experience with aprotinin, a nonspecific serine protease inhibitor, to determine whether it could ameliorate the inflammatory effects of cardiopulmonary bypass and improve outcome of single-ventricle palliation. METHODS Forty-six consecutive patients undergoing single-ventricle palliation using cardiopulmonary bypass were reviewed retrospectively. Aprotinin was used in 8 of 30 bidirectional cavopulmonary shunt and 10 of 16 Fontan procedures. RESULTS Aprotinin use was associated with a decrease in the early postoperative transpulmonary gradient among patients undergoing Fontan and bidirectional cavopulmonary shunt procedures. The bidirectional cavopulmonary shunt aprotinin group had a higher oxygen saturation and a decrease in quantity and duration of thoracic drainage. Among patients receiving aprotinin there were no episodes of mediastinitis, thrombus formation, or renal failure. CONCLUSIONS Aprotinin use in single-ventricle palliation was associated with decreased transpulmonary gradient and increased oxygen saturation consistent with decreased pulmonary vascular resistance. This retrospective study suggests that aprotinin has a favorable impact on the early postoperative course of single-ventricle palliation.