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Ann Thorac Surg 2007;84:2070-2075. doi:10.1016/j.athoracsur.2007.06.067
© 2007 The Society of Thoracic Surgeons

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Jason A. Williams
Lois U. Nwakanma
Nishant D. Patel
Diane E. Alejo
Vincent L. Gott
Luca A. Vricella
William A. Baumgartner
Duke E. Cameron
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Right arrow Congenital - cyanotic


Original Articles: Cardiovascular

Two Thousand Blalock-Taussig Shunts: A Six-Decade Experience

Jason A. Williams, MD, Anshuman K. Bansal, BS, Bradford J. Kim, BA, Lois U. Nwakanma, MD, Nishant D. Patel, BA, Akhil K. Seth, BS, Diane E. Alejo, BA, Vincent L. Gott, MD, Luca A. Vricella, MD, William A. Baumgartner, MD, Duke E. Cameron, MD*

Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland

Accepted for publication June 21, 2007.

* Address correspondence to Dr Cameron, Division of Cardiac Surgery, Pediatric Cardiac Surgery, The Johns Hopkins Medical Institutions, 600 N Wolfe St, Blalock 618, Baltimore, MD 21287 (Email: dcameron{at}csurg.jhmi.jhu.edu).

Presented at the Forty-third Annual Meeting of The Society of Thoracic Surgeons, San Diego, CA, Jan 29–31, 2007.


    Abstract
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
Background: The Blalock-Taussig shunt (BTS) remains valuable for palliation of congenital heart disease, but its role has evolved. We reviewed our total institutional experience with BTS to examine changes in its use and outcomes.

Methods: A retrospective review was performed of all patients undergoing BTS at our institution from November 1944 to May 2006. Hospital records and autopsy records were evaluated to determine patient demographics, diagnoses, operative data, hospital complications, and long-term outcomes.

Results: During the last 62 years, 2,016 BTS were performed by 28 surgeons on 1,880 patients from 35 countries. Classic BTS were performed in 75% (1,503 of 2,016 BTS). Diagnosis was tetralogy of Fallot in 72% (1,294 of 1,802), although diagnoses were imprecise in the early part of the series. Overall operative mortality was 14% (227 of 1,574). On follow-up, 32% of tetralogy of Fallot patients (411 of 1,294 patients) underwent subsequent total correction at our institution, and an additional 116 patients for whom follow-up was available had total correction of tetralogy of Fallot at other institutions, a combined total correction of tetralogy of Fallot rate of 41%. Of patients with complex congenital heart defects, 26% (106 of 404 patients) had subsequent cavopulmonary connection or atrial or arterial switch procedures. A comparison of the first and second halves of the series revealed several trends: decreasing mean annual number of BTS (66/year versus 9/year, respectively), decreasing operative mortality (16% versus 9%), and increasing proportion of single-ventricle diagnoses (5% versus 34%).

Conclusions: Evolution of the BTS has seen a decrease in overall use, particularly in tetralogy of Fallot, but greater application to single-ventricle cardiac lesions and improved operative survival.


    Introduction
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
On November 29, 1944, Dr Alfred Blalock performed the first successful palliation of a "blue baby" with pulmonic stenosis. Six months later, Drs Blalock and Taussig submitted a report of the first surgical series for the treatment of cyanotic heart disease involving pulmonary stenosis or atresia [1]. This landmark accomplishment was made possible by the collaboration of Alfred Blalock, Helen Taussig, and Vivian Thomas, and ultimately set the stage for rapid development of the field of cardiac surgery [2].

Since the 1940s, countless patients have benefited from the shunt procedure. Although the majority of patients had tetralogy of Fallot (TOF), this operation was quickly adapted to treat patients with a variety of cyanotic heart diseases, including tricuspid atresia, truncus arteriosus, transposition of the great vessels, and various forms of single ventricle [3–7]. Although operative techniques have evolved during the past six decades, outcomes continue to be excellent, and many patients are palliated indefinitely or until definitive repair can be undertaken.

Our institutional experience with Blalock-Taussig shunt (BTS) began with the first operation performed on Eileen Saxon in 1944. However, the indications and diagnoses for patients undergoing this procedure have changed markedly since then. More than 30 years have passed since we last reported our results with this procedure [6]. We sought to review our total institutional experience with BTS to identify changes that have occurred in its use and outcomes during the past six decades.


    Patients and Methods
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
Patient Selection
After obtaining institutional review board approval and waiver of consent, retrospective review was performed of all patients undergoing the BTS procedure at The Johns Hopkins Hospital from November 1944 through May 2006. All patients who underwent some form of systemic to pulmonary shunting procedure were included, regardless of whether this was a part of a larger procedure or set of procedures. The exceptions were patients undergoing central shunts, such as Potts or Waterston shunts. The list of patients was generated from a compilation of the cardiac surgery database, operative logs from the general operating room, and personal records and archives of Drs Blalock and Taussig, which are kept in the Division of Medical Archives at our institution. Patients in whom an anastomosis was found to be technically impossible at the time of thoracotomy or sternotomy before attempting the anastomosis were excluded from this study. However, patients were included who expired intraoperatively during an attempted anastomosis or who underwent an attempted anastomosis that was found to be technically impossible or inadequate.

Data Collection and Patient Variables
Information on patient demographics, diagnosis, type(s) of procedure(s), hospital complications, and long-term outcomes were gathered from medical records, medical archive documents, autopsy records, and direct patient contacts.

Statistical Analysis
Statistical analysis was performed using GraphPad Software (GraphPad Software, Inc, Del Mar, CA) and SPSS 12.0 software (SPSS Inc, Chicago, IL). Comparisons between groups were made using a two-tailed Student’s t test and Fisher’s exact test as indicated. All statistics are reported as mean ± standard deviation unless otherwise indicated. A probability value of less than 0.05 was considered significant. Patients with missing data points were excluded from analysis and reporting of those data, which accounts for the varied denominators throughout this report. For descriptive purposes, the early part of this series refers to 1944 through 1969 and the late part of the series refers to 1970 through 2006. These cutoffs were chosen arbitrarily to maintain whole decades within the groups, as well as to evaluate the era of BTS before and after the introduction of the modified BTS technique.


    Results
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
Patients
During the last 62 years, 2,016 BTS were performed by 28 surgeons on 1,880 patients from 35 countries. Data were available on 94.4% (1,774 of 1,880) of patients in this series. Diagnosis was tetralogy of Fallot (TOF) in 72% (1,294 of 1,802), although diagnoses were imprecise in the early part of the series. One hundred sixty patients (9%) had single-ventricle diagnoses and typically had BTS as part of a complex palliative procedure. The percentage of patients undergoing BTS for single-ventricle diagnosis increased significantly in the late part of the series (5% versus 34%; p < 0.0001). Tables 1 and 2 Go list the other pertinent demographic and clinical data for the patients in this series.


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Table 1 Demographic and Clinical Characteristics of Blalock-Taussig Shunt Cohort
 

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Table 2 Mean Age and Weight of Blalock-Taussig Shunt Cohort
 
Details of Procedures
Table 3 demonstrates the volume of BTS surgery performed each decade at our institution. Notably, the annual rate of BTS performed decreased significantly when the early part of this series was compared with the late part (66/year versus 9/year; p < 0.001).


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Table 3 Procedure Details by Decade
 
Of 2,016 BTS procedures performed during the study period, 75% (1,521 of 2,016) were classic BTS, using a direct anastomosis of either the right or left subclavian artery to the ipsilateral pulmonary artery. The modified BTS was introduced later in the series, and typically entails interposition of a Gore-Tex or Dacron graft between a branch pulmonary artery and either the subclavian artery (usually the one opposite to the aortic arch) or right brachiocephalic (innominate) artery. Figure 1 demonstrates the distribution of BTS procedures performed at our institution.


Figure 1
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Fig 1. Type of Blalock-Taussig shunt (BTS) procedures performed. (L = left; R = right.)

 
Of all the BTS procedures, 1,610 (80%) were performed using direct anastomoses of the patients’ native vessels. In addition to the classic BTS, other direct anastomoses included anastomosis of the innominate artery to the right or main pulmonary artery, and anastomosis of the right or left subclavian artery to the main pulmonary artery. Prosthetic interposition conduit (Gore-Tex) was used in 162 (8%) cases. Conduits used in the remaining procedures included saphenous vein (n = 27), internal mammary artery (n = 4), Dacron (4), subclavian artery homograft (n = 4), and Teflon (n = 3). The type of conduit used was unknown in 202 procedures (10%), but most were believed to be direct anastomoses (ie, classic shunts) as they occurred early in the series.

Early Outcomes
Overall operative mortality for all BTS patients was 14% (227 of 1,574). For those patients undergoing their first BTS, the operative mortality was 14% (220 of 1,595), whereas mortality for second BTS procedures was 11% (14 of 129). Six patients had a third BTS, but none died in the perioperative period. Operative mortality for all BTS procedures improved each decade (Fig 2), but did not reach statistical significance (p = 0.08).


Figure 2
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Fig 2. Operative mortality by decade.

 
Mean length of stay for all BTS in each decade are shown in Table 3. For patients undergoing their first BTS, mean postoperative length of stay was 16 ± 16 days. Patients undergoing a second BTS had a mean postoperative length of stay of 17 ± 16 days, whereas patients undergoing a third BTS had a mean postoperative length of stay of 15 ± 9 days.

Table 4 lists relevant complications after BTS compared by decade. Incidences are based on the number of patients with hospital course data available (1,574 patients). Of note, the incidence of prolonged intubation, sepsis, and wound infection all increased in the 1990s and 2000s. This most likely reflects the increased complexity of patients undergoing BTS during these decades.


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Table 4 Complications Exclusive of Mortality After Blalock-Taussig Shunt
 
Late Outcomes
On follow-up, 32% of TOF patients (411 of 1,294) underwent subsequent total correction of TOF at our institution. An additional 116 patients for whom follow-up was available underwent total correction of TOF at other institutions after BTS at our institution. The combined total correction of TOF rate was 41%.

Of patients with complex congenital heart defects, 26% (106 of 404) had subsequent cavopulmonary connection or atrial or arterial switch procedures. For the entire BTS cohort, 850 patients (45%) are known to have undergone multiple cardiac surgical procedures to palliate or correct underlying congenital cardiac lesions.

Actuarial survival of the BTS cohort is shown in Figure 3. This figure also demonstrates the number of patients for whom follow-up data were available in 10-year intervals after their original BTS. Of 710 patients known to have died after BTS, causes of death were known in 476 (67%) and are depicted in Figure 4. The most common cause of death after BTS was heart failure. Cardiac arrest, stroke, and brain abscesses were also common causes of late death. Other than heart failure, the most common cause of early mortality was complications as a result of hemorrhage, although no patient has died of hemorrhage in the last 30 years. Figure 5 depicts important trends in use and outcome of BTS during the past six decades: decreasing mean annual number of BTS, decreasing operative mortality, and increasing proportion of single-ventricle diagnoses.


Figure 3
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Fig 3. Kaplan–Meier cumulative survival.

 

Figure 4
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Fig 4. Causes of early and late death after Blalock-Taussig shunt for 710 patients known to have experienced early or late death after undergoing the procedure.

 

Figure 5
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Fig 5. Number of Blalock-Taussig shunt (BTS; gray bars) procedures, percentage of operative mortality (dashed line), and percentage of single-ventricle patients (solid line) by decade.

 

    Comment
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
In the early 1970s, Taussig and colleagues [3] first reported long-term results of BTS at our institution. The 12% operative mortality, combined with a 23% late mortality at 15 years, set the standard for surgical palliation of patients with TOF. Although results were inferior for patients with tricuspid atresia [4], truncus arteriosus [5], and transposition of the great vessels [6], the success with these disorders nonetheless demonstrated promise for treatment of many forms of cyanotic heart disease.

Many other groups subsequently demonstrated the effectiveness and safety of BTS for long-term palliation, regardless of patient age [8–11]. The success of the shunt stems from its high patency rate, technical ease of creation and takedown, low operative mortality, and low complication rate [9–11]. Once the BTS was demonstrated effective palliation for older children, attention turned to younger infants and neonates. During the past three decades, excellent results have also been seen in newborns and infants with cyanotic heart disease, with only modest increase in operative mortality in neonates and extremely low birth weight babies (<3 kg) [11–14]. These studies also demonstrated better survival when compared with the Waterston shunt [12].

In most series, patients with TOF tend to fare better than patients with other forms of cyanotic heart disease. Even after Lillehei and colleagues [15] reported total correction for TOF in 1954, surgeons continued to use the BTS for two-stage surgical repair of TOF with excellent results (>97% long-term survival) [16, 17]. The BTS is considered superior to other central shunts (Waterston or Potts) because of ease of takedown and less risk of pulmonary artery distortion and stenosis [18–20]. However, as surgical techniques improved and experience with total correction of TOF grew, surgeons have opted toward total correction earlier in life, leaving the BTS as an option for neonates, some patients with extremely low birth weight (<3 kg), those who are unstable at the time of presentation, or those who have anatomic issues that mitigate against early total correction [21, 22].

As utility of BTS in patients with TOF has waned, single-ventricle patients have become a more prominent group of patients undergoing shunting [23, 24]. Even though early reports of BTS outcomes when performed in conjunction with more complex reconstruction had operative mortalities near 30%, advancements in surgical techniques and perioperative management have yielded better outcomes in recent years. Survival after Norwood palliation using a BTS exceeds 90% in some centers [24, 25]. Our data demonstrate a significantly higher proportion of BTS recipients now carry a diagnosis of single ventricle. That operative mortality continues to improve despite the greater percentage of single-ventricle patients is a testament to improved care in recent years. The introduction of the right ventricle to pulmonary artery conduit (Sano modification) in Norwood operations has decreased overall use of BTS in this setting, but data are lacking on comparison of the results between the two approaches (BTS versus right ventricle to pulmonary artery conduit) [26–31].

Another important trend highlighted by our study is the modification in surgical technique to create a BTS. Developed in the 1970s, the modified Blalock-Taussig shunt using expanded polytetrafluoroethylene gained acceptance as an alternative to the classic BTS [32–34]. Most authors now consider the modified BTS to be the technique of choice because of four major advantages over the classic BTS: preservation of the subclavian artery; fewer technical problems with the anastomosis, including ease of insertion and takedown; greater pulmonary artery growth with less distortion of the pulmonary arteries; and lower shunt failure rate [35–37]. Median sternotomy is now our preferred incision, and the modified BTS has become the standard method for creating a systemic to pulmonary artery shunt in cyanotic infants [38–40]; indeed, median sternotomy with the use of polytetrafluoroethylene graft has been the standard technique at our institution since the early 1990s.

Like any retrospective study, our study has certain limitations. First, we lack standardized long-term follow-up on many of our patients. This results from the high percentage of patients from overseas countries and the lack of social security numbers to identify and track the American patients in the first three decades of the series. We also recognize that diagnoses were imprecise in the early part of this series, and many of the records from the first three decades have missing data that are unrecoverable.

Despite these limitations, we have shown trends in the utilization and technique of the BTS, namely that although fewer BTS are performed annually than in previous decades, operative mortality has fallen despite a higher percentage of patients with single ventricle and complex cyanotic heart disease. Our results with BTS continue to be excellent. This procedure remains valuable for the palliation of many types of congenital heart disease and enjoys an important position in the history of the evolution of our surgical specialty.


    Discussion
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
DR DENTON A. COOLEY (Houston, TX): Well, thank you, Dr Williams, for this interesting study, and it certainly brings to light some of the sequences and consequences of that early historic operation in November 1944. I was privileged to be on the original team and certainly cherish the memory. I have always believed that that was the dawn of the modern era of cardiac surgery, and I was privileged to be present to witness the beginning. It has been interesting to see the evolution and changes technically in the procedure. The concept, of course, has remained pretty much the same, that is, a systemic-to-pulmonary shunt. I think the big revolution has come in the last 20 years with the development of synthetic graft prostheses, which make it possible for the surgeon to select not only the length but also the diameter of the shunt and to try to estimate and predict the volume of the shunt involved. I know in some of our early trepidations we had much difficulty in all of those respects. For example current techniques do not require sacrifice of brachiocephalic tributaries.

I have noticed in our own Texas Children’s Hospital that there is an ongoing use of the modified Blalock-Taussig shunt. I think it is probably a larger series than you report from Hopkins, but it is an integral part of our palliative treatment of congenital heart disease. It is interesting to me to also note that it may even have use as a destination therapy. Just last week I saw a patient that I had operated on in 1956 with a classic Blalock-Taussig operation for tricuspid atresia. This patient has gone through birthing deliveries on two occasions uneventfully and now is a very active grandmother; however, she is facing the possibility of a cardiac transplantation. Nevertheless, the classic Blalock shunt is still continuing to function. So some of these patients with very complex congenital anomalies, particularly single-ventricle anomalies, where there is such a strong effort to convert every one of them to a Fontan type of physiology, some of them probably could enjoy a reasonably comfortable life with nothing more than the modified Blalock-Taussig operation.

Thank you very much Dr Williams and your colleagues at Hopkins, and I compliment you on this very interesting presentation.

DR WILLIAMS: Thank you for your kind comments.


    Acknowledgments
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
The authors would like to thank Andrew Harrison, Nancy McCall, Phoebe Evans Letocha, and Marjorie Winslow-Kehoe at the Department of Medical Archives at the Johns Hopkins Medical Institutions for their assistance with this manuscript. We are also indebted to Barbara Dobbs and Barbara Fleischman in the Cardiac Data Center for their technical assistance. This study was supported by the Joyce Koons Family Cardiac Endowment Fund, the Mildred and Carmont Blitz Cardiac Research Fund, the Irene Piccinini Cardiac Surgery Research Endowment, and the Hugh R. Sharp Cardiac Surgery Research Fellowship.


    References
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 

  1. Blalock A, Taussig HB. The surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia JAMA 1945;128:189-202.
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M. J. O'Connor, C. Ravishankar, J. A. Ballweg, M. J. Gillespie, J. W. Gaynor, S. Tabbutt, and T. E. Dominguez
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[Abstract] [Full Text] [PDF]


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Jason A. Williams
Lois U. Nwakanma
Nishant D. Patel
Diane E. Alejo
Vincent L. Gott
Luca A. Vricella
William A. Baumgartner
Duke E. Cameron
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