Experiences of parents of children with hypoplastic left heart syndrome during their treatment: a qualitative study.

AIM: To investigate the opinion and personal experience of parents of children born with Hypoplastic Left Heart Syndrome and what advice they would give to other parents who have to decide between treatment options. METHODS: We conducted a qualitative, descriptive and retrospective study by means of a survey directed to parents of children born with Hypoplastic Left Heart Syndrome in a tertiary hospital in Buenos Aires (Argentina). Their answers and data regarding medical procedures were analysed. RESULTS: Parents of thirteen out of sixteen patients with Hypoplastic Left Heart Syndrome were surveyed. Norwood surgery had been performed in all the patients, many had received other procedures, and five had died. In relation to the decision-making process, sixty-one percent of parents would recommend other parents to remain at peace after having done everything possible and 54% would suggest to not feel guilt despite the final result. None of the parents would recommend rejecting surgical treatment and choosing comfort care. CONCLUSION: The majority of parents of children with Hypoplastic Left Heart Syndrome would recommend continuing with the therapeutic effort in order to feel at peace and reduce feelings of guilt.

Advocacy at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.

The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.

Refining the Nasal Dorsum with Free Diced Cartilage.

Refining the nasal dorsum has become a major challenge in modern rhinoplasty as irregularities of the nasal dorsum account for a significant number of revision surgeries. In our department, free diced cartilage is now routinely applied for smoothening of the nasal dorsum. In this retrospective study, the outcomes with regard to irregularities or contour deficits of the nasal dorsum of 431 rhinoplasty cases operated by a single surgeon between July 2013 and June 2015, using free diced cartilage, are compared with 327 cases operated by the same surgeon between January 2007 and December 2008, before the introduction of the free diced cartilage technique. A decrease in early revision surgeries (i.e., revision within the 2-year period evaluated) due to dorsal irregularities or contour deficits is seen. Being a quick, easy, and highly cost-effective procedure, we feel that free diced cartilage is currently the ideal technique for refinements of the nasal dorsum.

Skin-fat-graft: a simple tool for reconstruction of small deep defects of the nose.

Small deep defects of the nose after resection of benign or malignant skin tumors are a common challenge in facial plastic surgery daily routine. The use of local flaps has several disadvantages for what reason they are a minor or no option especially in certain localizations in the lower third of the nose. Many elder patients suffer from comorbidities where complex more-staged reconstructional procedures drop out. We present a technique of nasal defect closure with a skin-fat composite graft. Between April 2010 and July 2013, we treated 42 patients with a total of 46 skin-fat-grafts to the nose. We reevaluated the esthetic and functional outcome in a retrospective analysis. In 80% of the cases, the results were rated excellent to satisfactory, 20% fair to poor. Reasons for worse validation were color and contour differences of grafts and surrounding tissue as well as alar retraction in very few cases. Nevertheless, we consider skin-fat-grafts to be a useful tool in single-layer nasal reconstruction in defects of smaller size.

Living to Tell the Tale: A First-Person Chronicle of the Development of the Fontan-Kreutzer Procedure.

The current surgical approach for patients with a single ventricle is the culmination of decades of dedicated research and groundbreaking innovation. From 1971 to the present day, a series of incremental advancements have significantly extended the life expectancy of these patients. Since the very beginning, Dr Guillermo Kreutzer and his team have pioneered different techniques with the ultimate goal of improving outcomes for these individuals. This is, narrated by him, the story of how it all began.

Fifth "Jatene Lecture on Surgical Innovation": Innovation in Congenital Heart Surgery: Contributions From South America.

We present the fifth "Jatene Lecture on Surgical Innovation" on Innovation in Congenital Heart Surgery, given at the Eighth Scientific Meeting of the World Society for Pediatric and Congenital Heart Surgery and Eighth World Congress of Pediatric Cardiology and Cardiac Surgery in Washington DC in 2023. We highlight what surgical innovation is and how innovation was accomplished in cardiac surgery and particularly in congenital heart surgery. A brief history of the development of congenital heart surgery across the world is summarized and we finally illustrate the South American contributions to congenital heart surgery, acknowledging the great innovations of Adib Jatene and Guillermo Kreutzer to our field.

Technical Aspects and Common Pitfalls of Norwood With Modified Blalock Taussig Shunt.

The Stage 1 Norwood procedure is the first of 3 stages in the surgical palliation of hypoplastic left heart syndrome and certain other single ventricle lesions with systemic outflow obstruction. In this article, we address some technical aspects and common pitfalls of the Norwood procedure with systemic to pulmonary shunt for HLHS palliation. We report our results with the Norwood with Blalock Taussig shunt in a cohort of 44 patients over a 7-year period in 2 institutions in Argentina. The results of the Norwood procedure have improved significantly through the understanding and refinement of the surgical techniques. Procedures must be technically perfect since residual lesions are poorly tolerated. Norwood with a modified Blalock Taussig shunt can be performed with low mortality and may provide excellent long-term outcomes.

Current Status of Training and Certification for Congenital Heart Surgery Around the World: Proceedings of the Meetings of the Global Council on Education for Congenital Heart Surgery of the World Society for Pediatric and Congenital Heart Surgery.

The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

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.

CABG With Internal Thoracic Artery in Children With Congenital Heart Defects: A Good Option When It Is the Only One.

OBJECTIVE: Coronary complications may present during or after repair of congenital heart defects. We report coronary artery bypass grafting (CABG) by internal thoracic artery (ITA) grafts to either coronary artery in children with congenital anomalies. METHODS: Four cases who underwent CABG with ITA grafts from March 2016 to March 2020 were retrospectively reviewed. RESULTS: At the time of operation, patient's ages and weight were 7 and 20 months old and 14 and 15 years old and 6.5, 10, 40, and 45 kg, respectively. Diagnosis were anomalous origin of the left coronary artery from the pulmonary artery with leftward lateral ostial origin (n = 1), neopulmonary annulus hypoplasia post arterial switch with contiguous right coronary artery (RCA) arising from the left facing sinus (n = 1), RCA stenosis after the Ross procedure (n = 1), and right coronary ostial obstruction after aortic valve replacement in truncus arteriosus (n = 1). Procedures included left ITA to left coronary ostium (n = 1), right ventricular outflow tract (RVOT) enlargement with pulmonary valve replacement with left ITA to RCA (n = 1), RVOT enlargement with pulmonary valve replacement with right ITA to RCA (n = 1), and aortic valve re-replacement, pulmonary valve replacement, and right ITA to RCA (n = 1). At last follow-up, all four patients were asymptomatic, with normal ventricular function, and all grafts were patent. CONCLUSIONS: The use of CABG in children is valuable alternative when dealing with complex coronary anatomy not suitable for classic repairs. In children, graft patency is required to be longer than 50 years; therefore, use of arterial grafts seems mandatory.

Lymphatic Decompression Concomitant With Fontan/Kreutzer Procedure: Early Experience.

OBJECTIVE: To present a strategy for identifying patients at risk of lymphatic failure in the setting of planned Fontan/Kreutzer completion, allowing a tailored surgical approach. METHODS: Since January 2017, clinical evaluation before performance of the Fontan/Kreutzer procedure included T2-weighted magnetic resonance imaging (MRI) lymphangiography. Thoracic lymphatic abnormalities were categorized using a scale of I to IV according to progression of severity. Patients with severe lymphatic abnormalities (types III and IV) underwent Fontan/Kreutzer with lymphatic decompression via connection of the left jugular-subclavian junction containing the thoracic duct to the systemic atrium (group A). RESULTS: Thirteen patients were enrolled. Magnetic resonance imaging showed type I abnormalities in four cases (30.7%), II in four (30.7%), III in two (15.3%), and IV in three (23.3%). Patients in types III and IV underwent a Fontan/Kreutzer with lymphatic decompression (group A, n = 5), while patients in types I and II underwent a fenestrated extracardiac Fontan/Kreutzer procedure without lymphatic decompression (group B, n = 8). Preoperatively, there were no differences in age, weight, ventricular dominance (right vs left), superior vena cava pressure, incidence of chylothorax after previous superior cavopulmonary anastomosis (Glenn), or need for concomitant procedures at Fontan/Kreutzer completion. There were no differences in procedural times between the groups, nor were there differences in mortalities and Fontan/Kreutzer takedowns. There were no statistically significant differences in early and late morbidity between the two groups with the exception of total volume of effusions output postoperatively. At median follow-up of 18 months (range, 4-28 months), all patients in group A are in New York Heart Association class 1 with no differences between groups in arterial oxygen saturation. CONCLUSIONS: Lymphatic decompression during Fontan/Kreutzer procedure was successfully performed in patients identified by MRI as predisposed to lymphatic failure. A larger cohort of patients and longer follow-up are required to determine the efficacy of this approach in preventing early- and long-term Fontan/Kreutzer failure.

The World Society for Pediatric and Congenital Heart Surgery: its mission and history.

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) was established in 2006 to assemble pediatric and congenital heart surgeons from all continents and regions of the world and their colleagues from related specialties dealing with pediatric and congenital heart disease. Since its birth, it has held a highly successful inaugural scientific meeting in 2007 in Washington, DC, and a World Summit on Pediatric and Congenital Heart Surgery Services, Education, and Cardiac Care for Children and Adults with Congenital Heart Disease in 2008 in Montreal. It currently has 549 members from 71 countries and in a short period of time has become the largest organization in the world of pediatric and congenital heart surgeons. Its brief history already seems to be a guarantee of a promising future. Projects in the areas of research, training and education, patient care, and community service will allow the Society to reach its goals. By bringing together professionals from every region of the world, the WSPCHS should play a significant role in the improvement of care for children and adults with congenital heart disease around the world.

The improvement of care for paediatric and congenital cardiac disease across the World: a challenge for the World Society for Pediatric and Congenital Heart Surgery.

The diagnosis and treatment for paediatric and congenital cardiac disease has undergone remarkable progress over the last 60 years. Unfortunately, this progress has been largely limited to the developed world. Yet every year approximately 90% of the more than 1,000,000 children who are born with congenital cardiac disease across the world receive either suboptimal care or are totally denied care.While in the developed world the focus has changed from an effort to decrease post-operative mortality to now improving quality of life and decreasing morbidity, which is the focus of this Supplement, the rest of the world still needs to develop basic access to congenital cardiac care. The World Society for Pediatric and Congenital Heart Surgery [http://www.wspchs.org/] was established in 2006. The Vision of the World Society is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The Mission of the World Society is to promote the highest quality comprehensive care to all patients with pediatric and/or congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with emphasis on excellence in education, research and community service.We present in this article an overview of the epidemiology of congenital cardiac disease, the current and future challenges to improve care in the developed and developing world, the impact of the globalization of cardiac surgery, and the role that the World Society should play. The World Society for Pediatric and Congenital Heart Surgery is in a unique position to influence and truly improve the global care of children and adults with congenital cardiac disease throughout the world [http://www.wspchs.org/].

Percutaneous Transhepatic Fontan-Kreutzer Completion of Hepatic Vein Inclusion.

We report the case of an 11-year-old girl with heterotaxy syndrome, dextrocardia, and azygos continuation of an interrupted inferior vena cava who had developed pulmonary arteriovenous fistulas after a Kawashima procedure consisting of bilateral superior cavopulmonary anastomoses. She presented with profound cyanosis, fatigue, and failure to thrive. An operative procedure to direct hepatic vein effluent to the pulmonary circulation was performed with placement of an extracardiac conduit between the hepatic veins and the left pulmonary artery. Persistence of cyanosis led to investigation, which led to the discovery of an unintentionally excluded right hepatic vein. A percutaneous transhepatic catheter intervention was performed in which a vascular plug was implanted to occlude the "missed" right hepatic vein, redirecting the flow through intrahepatic venovenous channels to the conduit. Clinical condition and arterial oxygen saturation were substantially improved one year after the two-step hepatic vein inclusion procedure.

History of the World Society for Pediatric and Congenital Heart Surgery: The First Decade.

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) is the largest professional organization in the world dedicated to pediatric and congenital heart surgery. The purpose of this article is to document the first decade of the history of WSPCHS from its formation in 2006, to summarize the current status of WSPCHS, and to consider the future of WSPCHS. The WSPCHS was incorporated in Canada on April 7, 2011, with a head office in Montreal, Canada. The vision of the WSPCHS is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The mission of the WSPCHS is to promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with an emphasis on excellence in teaching, research, and community service.

The Lymphatic System: The Achilles Heel of the Fontan-Kreutzer Circulation.

In spite of excellent long term survival the Fontan Kreutzer procedure commonly presents late failure due to end-organ damage. Several advances have been described to refine single ventricle management and surgical techniques. However, very little research has been dedicated to the lymphatic circulation in the precarious Fontan hemodynamic state. The lymphatic circulation is clearly affected since there is increased lymph production, which requires to be drained at a similar or higher pressure than it is produced, commonly resulting in chronic lymphedema. Chronic lymphedema induces fibrosis and end-organ failure even in normal circulation. Diverting lymph drainage to the low-pressured systemic atrium in Fontan may represent a valid alternative for the treatment of devastating complications as protein-losing enteropathy and plastic bronchitis and may prevent or decrease the development of end-organ fibrosis or failure.

Free Diced Cartilage: A New Application of Diced Cartilage Grafts in Primary and Secondary Rhinoplasty.

BACKGROUND: Irregularities or deformities of the nasal dorsum after hump reduction account for a significant number of revision rhinoplasties. The authors therefore developed a technique of meticulously dicing and exactly placing free diced cartilage grafts, harvested from septum, rib, or ear cartilage. The cartilage paste is used for smoothening, augmentation, or camouflaging of the nasal dorsum in primary or revision rhinoplasties. METHODS: A retrospective analysis of multisurgeon consecutive open approach rhinoplasties from January to December of 2014 was conducted at a single center. The authors compared the outcome of three different techniques to augment or cover the nasal dorsum after an observation period of 7 months. In group I, 325 patients with free diced cartilage grafts as the only onlay were included. In group II, consisting of 73 patients, the dorsal onlay was either fascia alone or in combination with free diced cartilage grafts. Forty-eight patients in group III received a dorsal augmentation with the classic diced cartilage in fascia technique. RESULTS: Four hundred forty-six patients undergoing primary and secondary rhinoplasties in which one of the above-mentioned diced cartilage techniques was used were included in the study. The authors found revision rates for dorsal irregularities within the 7-month postoperative observation period of 5.2, 8.2, and 25 percent for groups I, II, and III, respectively. CONCLUSION: The authors' findings strongly support their clinical experience that the free diced cartilage graft technique presents an effective and easily reproducible method for camouflage and augmentation in aesthetic and reconstructive rhinoplasty.

Augmentation of Bridging Leaflets in Repair of Atrioventricular Canal Defects.

Left atrioventricular (AV) valve regurgitation is the most common complication after a atrioventricular canal defect (AVCD) repair. Despite what appears to be a less complex repair, patients with partial and transitional AV canal have higher reoperation rates for left AV valve regurgitation and left ventricular outflow tract (LVOT) obstruction. Retraction of bridging leaflets with secondary attachments to the septal crest commonly produces increased tension and flattening of the medial left AV valve leaflet and LVOT obstruction after cleft closure. We describe a novel technique of detachment and patch augmentation of bridging leaflets to avoid these complications.