Reprint of: How He Did It - The James S. Tweddell Approach to Single Ventricle Palliation in Patients With Hypoplastic Left Heart Syndrome.

This article will detail Dr. James Tweddell's operative technique and considerations for 26 all three stages of SVP in patients with HLHS. This will include the Norwood Procedure with 27 Right-Ventricle-to-Pulmonary-Artery Conduit (Sano Modification), Bidirectional Glenn 28 (Superior Cavopulmonary Connection), and Extracardiac, Fenestrated Fontan Completion (Total 29 Cavopulmonary Connection). These techniques are the culmination of over 20 years of experience 30 and represent the final techniques he employed at both Children's Hospital of Wisconsin and 31 Cincinnati Children's Hospital Medical Center.

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.

What have we learnt 50 years after the first Fontan procedure?

The Fontan procedure is often the only definitive palliative surgical option for patients with a variety of complex CHD sharing in common, a single, dominant ventricle. In recent decades, imaging and therapeutic improvement have played a crucial role in those patients in whom many complications can hamper their life. After 50 years from the first procedure, heart transplantation remains the only definitive treatment for those with a failing Fontan circulation.

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.

The Fontan circulation after 45†years: update in physiology.

The Fontan operation was first performed in 1968. Since then, this operation has been performed on thousands of patients worldwide. Results vary from very good for many decades to very bad with a pleiad of complications and early death. A good understanding of the physiology is necessary to further improve results. The Fontan connection creates a critical bottleneck with obligatory upstream congestion and downstream decreased flow; these two features are the basic cause of the majority of the physiologic impairments of this circulation. The ventricle, while still the engine of the circuit, cannot compensate for the major flow restriction of the Fontan bottleneck: the suction required to compensate for the barrier effect cannot be generated, specifically not in a deprived heart. Except for some extreme situations, the heart therefore no longer controls cardiac output nor can it significantly alter the degree of systemic venous congestion. Adequate growth and development of the pulmonary arteries is extremely important as pulmonary vascular impedance will become the major determinant of Fontan outcome. Key features of the Fontan ventricle are early volume overload and overgrowth, but currently chronic preload deprivation with increasing filling pressures. A functional decline of the Fontan circuit is expected and observed as pulmonary vascular resistance and ventricular filling pressure increase with time. Treatment strategies will only be successful if they open up or bypass the critical bottleneck or act on immediate surroundings (impedance of the Fontan neoportal system, fenestration, enhanced ventricular suction).

Four decades of Fontan palliation.

The Fontan palliation was introduced in 1968 to treat cardiac malformations unsuitable for biventricular repair. This procedure has transformed the surgical management of congenital heart disease. In this Review, we reflect on the outcomes and clinical problems associated with this unique circulation after more than 40 years of experience. We also summarize the evolution of the Fontan procedure, highlight the long-term clinical issues and their management, and consider future expectations of a circulation driven by a single ventricle with the systemic and pulmonary blood flow in series rather than in parallel.

The Fontan/Kreutzer procedure at 40: an operation for the correction of tricuspid atresia.

The first atriopulmonary anastomosis (APA) with neither a valve in the inferior vena cava (IVC) nor an Glenn shunt was performed in 1971. A fenestration was intentionally left in the atrial septum. In a second patient, the APA incorporated the patient's own pulmonary valve, which had been removed from the outflow tract of the right ventricle. Since the early days, our rationale was that the right atrium would only function as a pathway, and the end diastolic pressure and the systole of the main ventricle would be the principal "pump" of this system. The late hemodynamic problems of the APA have decreased with newer and better surgical techniques, such as the lateral tunnel (LT) or the extracardiac conduit (EC). Although these procedures have improved the prognosis and quality of life of patients with a univentricular heart (UH), in the long run, deterioration frequently occurs because of chronic low cardiac output and high central venous pressure. Progressive increase in pulmonary vascular resistances and ventricular dysfunction are frequently the underlying reasons for this deterioration. However, such deterioration is not inevitable in every case, as shown in the longest survivor of the world after 34 years of follow-up. The Fontan Kreutzer (FK) palliation represents the best surgical option despite its uncertain late outcome. Certainly, it is one of the triumphs of cardiac surgery in congenital heart disease.

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.

Fontan operation and the single ventricle.

The Fontan operation has gone through multiple incarnations since Fontan and Baudet's initial description in 1971. Through the medical dossier of a patient with a single ventricle, we plot the history of medical, surgical, and percutaneous interventions over the past 40 years, specifically focusing on the Fontan procedure, its development, indications, sequelae, and complications. Cardiac computed tomography with angiography is highlighted as a noninvasive imaging tool for the evaluation of the complex Fontan circulation.