A thank you to the medical community

S. Bert Litwin, MD, medical director emeritus, Cardiovascular Surgery, Herma Heart Center, Children's Hospital of Wisconsin; clinical professor, Surgery (Cardiovascular), Medical College of Wisconsin.

S. Bert Litwin, MD

I will leave clinical practice of congenital heart surgery after 34 years at Children's Hospital of Wisconsin. My wife and I will spend winter seasons at our home in Florida and return to Milwaukee in the summers.

I want to sincerely thank all the fine physicians, nurses and other medical personnel in Wisconsin and the upper Midwest with whom I have had the honor to work. It has been a pleasure for me to help in the care of so many of your patients with congenital heart disease. Without your support for so many years our program could not have prospered as it did. I have enjoyed our association and look to so many of you as my personal friends.

I liken myself at this time to General Colin Powell as he writes in his book, "My American Journey." In the last chapter he describes his thoughts after the farewell parade in his honor on the occasion of his retirement after 35 years in the Army. "That night I took off my uniform for the last time. In the years I had worn it I benefited beyond my wildest hopes from all that is good in this country and I had overcome its lingering faults. I had found something to do with my life that was honorable and useful, that I could do well and that I loved doing.  That is rare good fortune in anyone's life. My only regret was that I could not do it all over again ... "

S. Bert Litwin, MD

Maryanne Kessel, RN, MBA, director, Herma Heart Center, Children's Hospital of Wisconsin.

This month we say farewell to S. Bert Litwin, MD, after more than three decades of caring for the children of Wisconsin. He has done this both through his skill as a leading cardiothoracic surgeon and as a philanthropist. From his hometown of Jasper, Ala., to home base of Children's Hospital of Wisconsin, Dr. Litwin helped thousands of children live with heart disease and defects.

Dr. Litwin earned his undergraduate degree at the University of Alabama and his medical degree from Harvard Medical School, Boston. He completed his clinical and research fellowship in Surgery at Harvard Medical School and Massachusetts General Hospital in Boston, with a postdoctoral fellowship at the National Heart Institute, National Institutes of Health.

In 1968 he became a clinical instructor in Surgery at Harvard Medical School and assistant in Cardiovascular Surgery at Children's Hospital Medical Center in Boston. In 1968 and 1969 he was certified by the American Board of Surgery and American Board of Thoracic Surgery.

In 1972, Dr. Litwin came to Milwaukee to accept positions as director of Cardiovascular Surgery at Children's Hospital of Wisconsin and associate professor of Surgery at the Medical College of Wisconsin. He has since served as chief of staff at Children's Hospital and professor of Surgery at the Medical College.
Before his arrival, most children with heart disease and defects simply could not be cared for at Children's Hospital of Wisconsin.

Dr. Litwin has continued to foster the growth of the program he began 34 years ago. He has held the position of medical director emeritus, Cardiothoracic Surgery, for the Herma Heart Center. The center was established in 2003 when Cardiology and Cardiothoracic Surgery were formally combined into one integrated center to provide seamless care for children with congenital heart disease and their families. Herma Heart Center now is recognized as a leading pediatric heart program in the nation. The center's excellence in heart care is one of many factors that has led Children's Hospital of Wisconsin to be recognized nationally as well, thanks to the foundation of the cardiac program developed by Dr. Litwin.

Dr. Litwin's dedication to the children who turn to Children's Hospital of Wisconsin for care goes far beyond the operating room. He has been an advocate and champion of all aspects of growth of the Children's Hospital and Health System as it has expanded care throughout the state and region. We wish him well. He will be dearly missed. 

Cardiac MRI for the assessment of tetralogy of Fallot

Margaret M. Samyn, MD, FAAP, FACC, pediatric cardologist, Herma Heart Center, Children's Hospital of Wisconsin; assistant professor, Pediatrics (Cardiology), Medical College of Wisconsin.

Tetralogy of Fallot, the most common cyanotic congenital heart disease, includes overriding aorta, right ventricular hypertrophy, conotruncal ventricular septal defect, and sub pulmonary stenosis or pulmonary atresia (also known as right ventricular outflow tract (RVOT) obstruction). Although survival of patients undergoing surgical repair of tetralogy of Fallot (TOF) has improved steadily, the majority of patients have residual hemodynamic abnormalities. Relief of RVOT obstruction often involves placement of a patch, as well as a pulmonary valvotomy. Repair might also involve the placement of an RV to PA conduit (e.g. homograft), especially in patients with TOF and pulmonary atresia or in patients with a major coronary artery crossing the RVOT. These interventions may result in pulmonary valve regurgitation (PR) and, later, progressive right ventricular (RV) dilatation and dysfunction, with resulting decreased exercise capacity and/or arrhythmia. Furthermore, conduit stenosis and regurgitation invariably occur, often requiring reoperation or transcatheter intervention.

In these TOF patients with residual hemodynamic abnormalities, clinical decision making, especially the decision to perform pulmonary valve replacement, often is influenced by measurements of pulmonary regurgitation, as well as right ventricular size and function. Today, cardiac magnetic resonance (CMR) generally is accepted as the technique of choice for making these measurements.

Table 1: Limitations of echocardiography

1. Poor echocardiographic windows with age, increased body habitus, or post operative scar tissue. 2. Geometric assumptions for RV volume calculations. 2. Limited imaging of anterior structures. 3. Limited imaging of branch pulmonary arteries. 4. Qualitative assessment of pulmonary regurgitation (PR). 5. Poor agreement of Doppler peak instant. gradient compared with catheter peak-to-peak gradient (frequent over estimation by Doppler).

Post operative anatomic detail may not be well seen by traditional non-invasive imaging (i.e. echocardiography, ECHO). As a patient ages, due to poor echocardiographic windows resulting from increased body habitus or presence of postoperative scar tissue, ECHO may have limited utility in the adolescent and young adult patient (Table 1).

CMR aids with assessing cardiac anatomy, including the caliber of branch pulmonary arteries, the presence of aorto-pulmonary collateral vessels and the associated RVOT abnormalities (Figures 1 & 2).

Figure 1: Postoperative patient with tetralogy of Fallot with VSD patient, large RVOT aneurysm, RV dilation, and trace aortic regurgitation.

Furthermore, serial CMR, employed during the long-term follow-up of patients with repaired tetralogy of Fallot (TOF), will allow progressive assessment of cardiac chamber dimensions and function. Using standard CMR pulse sequences (i.e. white blood techniques like gradient echo or steady state free precession, (SSFP) imaging), the well-constructed CMR protocol uses a short axis plane to characterize the end-diastolic and end-systolic volume of the left ventricle (LV) and the difficult to evaluate right ventricle (RV), which typically is not well seen by echocardiography due to its anterior location. MRI offers a reproducible means of volumetric data acquisition, validated for both adults and children. By using velocity encoded cine (VENC) CMR sequences (also known as phase contrast cine MR), the cardiologist also can obtain a better understanding of the degree of pulmonary regurgitation (Figure 3).

Figure 2: Patient with repaired tetralogy of Fallot and CMR showing right pleural effusion, dilated RV, tricuspid valve regurgitation, and hypoplastic right pulmonary artery.

To verify the amount of pulmonary regurgitation determined by VENC CMR, if no other major source of regurgitation or shunt exists, RV and LV stroke volumes (calculated by the formula end-diastolic volume (EDV) minus end-systolic volume (ESV)) should be equal; differences in stroke volumes reflect the added regurgitant volume.

Deciding when to re-intervene for adolescents and young adults with repaired TOF, who have residual defects (especially PR, RVOT obstruction, RVOT aneurysm, RV dilation, and RV dysfunction) is a complicated matter. The cardiologist and cardiothoracic surgeon must take into account a clear assessment of symptoms, cardiac anatomy, and hemodynamic data (ventricular function and vascular flow). Objective measures of the patient's symptoms are readily available (i.e. Ross or New York Heart Association classification of congestive heart failure combined with VO2 max Bruce protocol exercise treadmill testing). Now, with the increased use of CMR, the limitations of echocardiography (especially regarding serial quantification of RV dimensions and function, as well as pulmonary regurgitation) can be overcome. Well-constructed, prospective clinical trials to assess best timing of re-intervention in this growing post-operative tetralogy of Fallot population can now be planned using this robust non-invasive imaging modality, CMR.

Set up imaging plane perpendicular to flow to generate this image. Post process cine image - tracing circumference of main pulmonary artery at different times in the cardiac cycle.

Figure 3: Graphic display of main pulmonary artery (MPA) flow volume, as determined by velocity encoded (VENC) CMR, at different times in the cardiac cycle. Integration of the area under the curve gives volume to allow determination of pulmonary regurgitant fraction (51 ml/124 ml x 100 = 41 percent.)

Late follow-up of tetralogy of Fallot

Peter Bartz, MD, cardiologist, Herma Heart Center, Children's Hospital of Wisconsin; assistant professor, Internal Medicine and Pediatrics in the divisions of Adult Cardiovascular Medicine and Pediatric Cardiology, Medical College of Wisconsin.

Figure 1: Tetralogy of Fallot

In 1988, Etienne-Louis Fallot correlated the clinical finding of cyanosis with the four anatomical findings that are known as tetralogy of Fallot (TOF) (Figure 1). The natural history of this malady is poor. Left untreated, approximately 25 percent of children with this defect die in infancy, 40 percent die before age 3, and 70 percent die before age 10. In 1945, the first systemic-to-pulmonary shunt for palliation of cyanosis in TOF was performed. Approximately 10 years later, the first intracardiac repair was accomplished. Today, palliative shunting is performed only in severely ill infants for whom complete repair is unsuitable. Elective complete surgical correction (closure of the ventricular septal defect and relief of right ventricular outflow obstruction) typically is performed between 4 to 8 months of age. Survival of patients who have had operative repair is excellent. Surgical mortality is less than 3 percent in children and 2.5 to 8.5 percent in adults. Long-term survival rates in patients 30 years after repair have been reported to be greater than 85 percent.

Table 1: Known long-term complications of tetralogy of Fallot

1. Endocarditis.  2. Aortic regurgitation. 3. Left vetnricular dysfunction.  4. Right ventricular dysfunction.  5. Residual pulmonary regurgitation.  6. Residual right ventricular outflow tract obstruction.  7. Exercise intolerance.  8. Atrial tachyarrhythmia.  9. Ventricular tachyarrhythmia.  10. Heart block.  11. Sudden cardiac death.

Despite the improvement in survival, late symptoms of tetralogy of Fallot occur and can range from mild exercise intolerance to sudden cardiac death (Table 1).
Complications typically are attributed to the intrinsic characteristics of TOF or sequelae of the surgical repair. Aortic regurgitation may be due to aortic annulus dilation or damage to the valve during ventricular septal defect closure. Prolonged palliation with systemic-to-pulmonary shunts results in chronic left ventricular volume overload, which may cause left ventricular dysfunction. Inadequate myocardial protection during cardiopulmonary bypass can result in left or right ventricular dysfunction. Depending on the surgical technique, individuals may be left with residual right ventricular outflow obstruction or clinically significant insufficiency. Atrial flutter and atrial fibrillation are relatively common in the adult with previous TOF repair. Up to 60 percent of patients with repaired TOF may have nonsustained ventricular arrhythmias; this symptom most likely is to occur in patients who are older at the time of surgical repair and those with moderate or severe pulmonary regurgitation, systolic and diastolic ventricular dysfunction, prolonged cardiopulmonary bypass, or prolongation of the QRS interval (>180 msec). Late sudden death from cardiac causes has been reported to occur in up to 6 percent of patients.

Reintervention for late symptoms of TOF is necessary in at least 20 percent of patients. When indicated, either cardiac surgery or interventional catheterization can be an effective treatment. One reason for reintervention is pulmonary regurgitation. This may develop as a consequence of surgical repair of the right ventricular outflow tract. Although substantial regurgitation can be tolerated for long periods, enlargement of the right ventricle eventually occurs with resultant right ventricular dysfunction, which may require replacement of the pulmonary valve. Another reason is aneurysm formation at the site of the right ventricular outflow tract repair. Although such aneurysms  usually are identified incidentally, rupture has been reported in rare cases.

Because most adolescents and adults with previous surgical treatment of TOF enjoy a relatively symptom-free lifestyle, some receive only sporadic medical care. However, the 32nd Bethesda Conference Task Force recommends frequent follow-up, generally every 12 to 24 months. Such evaluations should include a detailed history and clinical examination. Diagnostic studies should be standardized, usually including electrocardiogram, chest x-ray, echocardiogram, exercise testing and holter monitoring. The need for more extensive evaluations (e.g., cardiopulmonary stress testing, cardiac MRI, cardiac catheterization) depends on the individual patient's clinical course and findings. Part of regular follow-up should include the detection of any new or progressive cardiac symptomss and patient education.

Exercise recommendations for patients with TOF repair must be based on residua and sequelae from previous interventions. All patients with TOF require lifelong endocarditis prophylaxis. Pregnancy risk is low in women who have had successful correction of TOF and who have good underlying hemodynamics. However, long-term symptoms such as residual shunt, right ventricular outflow tract obstruction, arrhythmias, pulmonary regurgitation, right ventricular systolic dysfunction, pulmonary hypertension (caused by the effects of a previous palliative shunt), or left ventricular dysfunction (caused by previous volume overload) increase the likelihood of pregnancy complications and require independent consideration.

Over the past 50 years, there has been a significant improvement in the early and late outcome for patients with TOF. However, morbidity remains substantial. Meticulous long-term medical management at centers specializing in the care of congenital heart disease is mandatory. To diagnose and care for this complex patient group, Children's Hospital of Wisconsin has established the Adult Congenital Heart Disease Clinic as part of the Herma Heart Center. The goal of this program is to provide state-of-the-art comprehensive diagnostic and interventional care for adults with congenital heart disease. For appointments or referrals, call (414) 266-6784.

Scimitar syndrome

L. Eliot May, PA-C, Cardiothoracic Surgery, Herma Heart Center, Children's Hospital of Wisconsin.

Scimitar syndrome is a rare congenital anomaly in which all the right pulmonary veins or occasionally the veins draining the right lower and right middle lobes enter the inferior vena cava either just above or below the diaphragm. The name "scimitar" is derived from the characteristic "Turkish sword" appearance of the anomalous pulmonary venous channel seen on chest radiograph. This malformation is frequently associated with other anomalies including hypoplasia of the right lung with bronchial abnormalities and pulmonary sequestration, anomalous arterial supply to the affected lung from the descending aorta, rightward shifting of the mediastinum, and intracardiac defects including VSDs and tetralogy of Fallot.

Older children and adults with Scimitar syndrome typically present with asymptomatic heart murmurs, recurrent respiratory infections or mild exertional dyspnea. Newborns and infants with severe disease present with varying degrees of cyanosis, respiratory distress, congestive heart failure and pulmonary artery hypertension. Treatment of Scimitar syndrome is required in symptomatic patients and typically involves surgical redirection of the anomalous venous drainage to left atria, ligation/embolization of vascular supply to the sequestered lobe and pneumonectomy.

Figure A: Chest x-ray demonstrating scimitar vein shadow highlighted by arrows. Figure B: MRI cross-section demonstrating dilated right ventricle and scimitar vein draining to dilated inferior vena cava. Figure C: MRI longitudinal image again demonstrating SV drainage of the right pulmonary artery to IVC at the level of the diaphragm. Figure D: 3D MRI reconstruction of scimitar syndrome. PA to right lung with complete anomalous pulmonary venous return to SV that drains to the IVC.

Surgical technique
Achievement of unobstructed drainage of pulmonary venous return to the left atrium is the goal of surgical intervention. In addition, the anomalous systemic arterial supply to the abnormal pulmonary lobe(s) is ligated or embolized. Associated intracardiac defects are also addressed at the time of surgery. Surgical approaches vary depending upon the patient's individual anatomy and associated defects. When Scimitar syndrome is not associated with intracardiac defects, a right thoracotomy with direct reimplantation of the anomalous pulmonary vein to the left atrium can be achieved in some cases without the need for cardiopulmonary bypass. When direct anastomosis of the anomalous pulmonary vein to the left atrium is not possible, or when intracardiac defects are present, cardiopulmonary bypass is utilized. In complex cases the Scimitar vein is anastomosed to the right atrium and an intracardiac baffle or patch is constructed to redirect the Scimitar vein drainage to the left atrium. In cases with marked pulmonary parenchymal changes and/or severe hypoplasia of the right lung, partial or complete pneumonectomy is required.

Postoperative considerations
The postoperative course following repair of Scimitar syndrome is related to the degree of congestive heart failure, respiratory compromise and pulmonary artery hypertension encountered preoperatively. Older patients who present with mild symptoms typically have an uncomplicated recovery. Infants presenting with congestive heart failure or severe pulmonary artery hypertension typically have a more involved postoperative course. Potential surgical complications include phrenic nerve injury with resultant diaphragm paralysis, thoracic duct injury and postoperative bleeding.  Prolonged mechanical ventilation may be required for infants who present with severe symptoms. Invasive monitoring and vasoactive infusion requirements along with length of hospital stay vary. 

Long-term prognosis
The age at detection of scimitar syndrome and the presence of associated anomalies is important in predicting outcomes. In general, infants presenting in heart failure have a greater number of associated anomalies and their prognosis is guarded. The prognosis for older children and adults diagnosed with Scimitar syndrome is much more benign and normal activity and life span are expected. Therefore the therapeutic approach for patients with scimitar syndrome diagnosed beyond the neonatal period should be more conservative.

Clinical director of Cardiac Critical Care named

Nancy Ghanayem, MD

Nancy Ghanayem, MD, has been appointed clinical director of Cardiac Critical Care at Children's Hospital of Wisconsin. With leadership of Tom Rice, MD, medical director of Critical Care, Dr. Ghanayem will help develop, organize and monitor the quality and outcomes of cardiac clinical operations in the intensive care unit.

Dr. Ghanayem received her medical degree from Rush University in Chicago. She completed a residency in Pediatrics and a fellowship in Pediatric Critical Care at the Medical College of Wisconsin and Children's Hospital. She completed additional cardiac intensive care training at Children's Hospital of Philadelphia and at the Royal Children's Hospital in Australia.

2006 Statistics through November