Extremely Long-Term Follow-Up of Dr William Glenn's Original Patient Cohort With Superior Cavopulmonary Anastomosis (1958-1990).

BACKGROUND: Historically, Dr William Glenn performed the first classic superior cavopulmonary anastomosis in a seven-year-old child at Yale in 1958. By 1990, this operation was performed consecutively in over 90 patients. With over 60 years of follow-up, this is the longest survival record of early Glenn patients from the first 30 years. METHODS: We performed a single center, retrospective evaluation of patients undergoing a Glenn operation. A collected list of surviving patients, previously updated in 1988, included demographics, age at procedure, and underlying diagnosis. Follow-up data were obtained in May 2022 using electronic medical records to determine survival, age of survivors, and age of deceased. RESULTS: Ninety-five patients underwent the Glenn operation from 1958 to 1990: 58.9% (n = 56) were male and 41.1% (n = 39) female. Fifteen patients were lost to follow-up, but 12 were alive in 1988. Sixty patients were deceased (68.1%), with an average age of 33.5 ± 18.3(range, 2-78, excluding seven early deaths) years. The oldest patient who passed away was a 78-year-old male with tetralogy of Fallot. Twenty patients remain alive, with an average age of 47.5 (range, 32-66) years. Four patients who are still alive today (20% survivors) are older than 60 years. CONCLUSIONS: Since Dr Glenn's original operation, the technique, timing, and indications have been modified (ie, bidirectional Glenn) to adapt to the current era. By following this initial group of patients, we can approach completion of the survival rates for adult congenital patients who were some of the first pediatric patients to receive this ground-breaking palliative procedure.

A Tribute to the Pioneers of Right Heart Bypass: An Historical Review.

Cavopulmonary anastomosis was first described by Carlon, Mondini, De Marchi in a canine model in 1951 and later, in the clinical practice, by Glenn in 1958. Total right heart bypass was first introduced by Fontan and Kreutzer in 1971, in each instance as treatment for tricuspid atresia. Several modifications of such a procedure followed the initial concept of the right atrium as a pumping chamber, including modifications aimed to minimize energy loss at the anastomotic level and arrhythmias. Tribute is given to our pioneers who developed such an operation aimed to treat any child with functionally univentricular hearts.

The Superior Cavopulmonary Connection: History and Current Perspectives.

The development of the superior cavopulmonary connection is a rich illustration of international influences in congenital cardiac surgery. The bidirectional Glenn and hemi-Fontan procedures have improved survival as both definitive and staged functional single ventricle palliation. The optimal timing of the second-stage superior cavopulmonary procedures varies by center but for low- and intermediate-risk patients, this may be within three to six months after the Norwood procedure. The list of risk factors continues to grow but the most frequently cited factors include atrioventricular valve regurgitation, decreased ventricular function, need for reintervention, and failure to attain nutritional and growth goals. Ongoing prospective, multi-institutional studies, particularly those fostered internationally by the World Society for Pediatric and Congenital Heart Surgery and other associations, will hopefully provide further clarification of the complex management issues related to patients with functional single ventricle physiology.

Quality of Life for Historic Cavopulmonary Shunt Survivors.

BACKGROUND: Beginning with Dr William Glenn in 1958, 90 patients with congenital heart lesions underwent cavopulmonary (Glenn) shunts over a 30-year period. In 2015, the follow-up data on this original cohort were reported. The study focuses on the current quality of life of this cohort. METHODS: Of the original 91 cavopulmonary shunt survivors, 14 (ages: 26-59; average length of postsurgical follow-up: 38.4 years) completed the Rand 36-Item Short-Form Health Survey, volume 2 (SF-36v2) quality-of-life survey at clinical office visits, over the phone or via regular mail. Diagnoses included tricuspid atresia (n = 9), double inlet left ventricle (n = 1), d-transposition of great arteries (n = 1), Ebstein's anomaly (n = 1), tetralogy of Fallot (n = 1), and pulmonary atresia with hypoplastic right ventricle (n = 1). Norm-based comparison of cavopulmonary shunt survivors to the general population was performed using a one-sample t test. RESULTS: The 36-question health survey, SF-36v2, provided physical and mental health summary measures standardized to achieve a mean of 50. The 14 survivors' physical composite score was 47.18 (standard deviation [SD]: 8.24; P = .22) and mental composite score was 52.71 (SD: 5.64; P = .095). CONCLUSION: The cavopulmonary shunt is now a widely used surgical palliation for single-ventricle patients and can be associated with physical and mental health outcomes similar to the general US population. Cavopulmonary shunt survivors' SF-36v2 survey results do not demonstrate a statistically significant difference from the general US population in physical or mental health measures. The results of this study contribute to the discussion of quality of life for patients with congenital heart disease by following up with the oldest known survivors.

Dr Helen Taussig (1898 1986): pioneering American paediatric cardiologist.

In 1944, Helen Taussig in collaboration with Alfred Blalock initiated the first surgical treatment of cyanotic congenital malformations of the heart. She may be regarded as the founder of paediatric cardiology.

A prelude to Fontan.

In the decades preceding the Fontan operation, there was an intensive experimental and clinical quest to bypass the right heart. Whereas right heart bypass was successfully achieved in animal experiments, clinically only partial bypass (superior cava-right pulmonary artery anastomosis) was applied successfully. This intensive experimental and clinical activity provided the background for the Fontan operation.

Has the time come to rename the Blalock-Taussig shunt?

OBJECTIVE: To describe the contribution of Vivien Thomas to the scientific and technical development of the Blalock-Taussig shunt. DESIGN: Review of the literature and of the papers of Vivien Thomas, Alfred Blalock, and Helen Thomas. Literature review was done using MEDLINE, Ovid, and Library of Congress databases for topics including Vivien Thomas, Alfred Blalock, Helen Taussig, and tetralogy of Fallot. Bibliographies of articles were also searched. SETTING: The papers of Thomas, Blalock, and Taussig were reviewed at the Alan M. Chesney Medical Archives at the Johns Hopkins Medical Institutions. PATIENTS/SUBJECTS: None. INTERVENTIONS: None. RESULTS: Vivien Thomas was a primary contributor in both the scientific basis for and the surgical technical aspect of the Blalock-Taussig shunt. He did much of the fundamental studies in Dr. Blalock's laboratory by himself. Blalock was able to do only one practice procedure in the animal model before performing the first surgery in a 15-month-old girl with tetralogy of Fallot. Thomas stood behind Blalock during the procedure to provide advice to Blalock. CONCLUSIONS: Thomas' essential contributions to the development of the Blalock-Taussig shunt remain obscured by the stature of Alfred Blalock and Helen Taussig. This oversight should be corrected.

Cavo-pulmonary shunt: from the first experiments to clinical practice.

The cavo-pulmonary anastomosis is often referred to as Glenn shunt today. The concept of cavo-pulmonary shunting, however, was developed independently by many surgeons. While the work of some of them is widely recognized, the pioneering contributions of many others fall into oblivion. Nonetheless, each of them contributed something original and precious to the total sum of our modern knowledge. It seems timely, as we enter the new millennium, to give due credit to those individuals who put their minds and efforts into helping sick children. These people deserve recognition.