The role of subcoronary implantation technique in children and young adults undergoing Ross operation

Objectives: The aim of this work was to report our experience with the modified subcoronary autograft implantation in children and young adults and to furthermore discuss the outcomes and role of this technique in growing patients requiring Ross operation. Methods: All patients who underwent modified subcoronary Ross/Ross-Konno operation at our institution were included. Early and late survival, morbidity, reinterventions, aortic valve function, and aortic root dimensions were observed in a retrospective manner. Results: During the study interval from January 2013 to January 2019, a total of 50 patients received the modified subcoronary Ross/Ross-Konno operation. The median age at surgery was 6.3 years (interquartile range [IQR]: 1.4 - 13.6, 58% males), including ten infants (20%). During a median follow-up of 31.2 months (IQR: 14.4 - 51), there was one early and one late death with an overall survival at 5 years after surgery of 95%. The autograft had to be replaced in two patients, 11 and 15 months after their initial Ross operation. Freedoms from reoperation on the autograft and/or on the right ventricle to pulmonary artery conduit were 94% and 97% at 5 years, respectively. Freedom from aortic valve regurgitation > mild was 97% at 5 years. Aortic root diameters at all levels remained in normal ranges at last follow-up. Conclusion: The modified subcoronary Ross/Ross-Konno operation in children and young adults showed excellent early and mid-term outcomes. Our modification of the autograft implantation technique proved to be reproducible. An even broader use of this technique in children might be advantageous in order to avoid situations when prosthetic supporting techniques are precluded due to somatic growth.ZUSAMMENFASSUNG Hintergrund: Das Ziel dieser Arbeit war es über unsere Erfahrungen mit der Modifikation der subkoronarer Implantation des Autograft bei Kindern und jungen Erwachsenen während einer Ross-Operation zu berichten. Weitergehend soll das Outcome sowie die Rolle dieser Modifikation in der Behandlung von Patienten während des körperlichen Wachstums soll diskutiert werden. Methoden: In einer retrospektiven Studie an 50 von Januar 2013 bis Januar 2019 am Deutschen Herzzentrum Berlin konsekutiv mittels Ross/Ross-Konno-Operation in subkoronarer Modifikation behandelter Patienten, wurden die primären und sekundären Endpunkten wie Früh- und Spätmortalität, Morbidität, Reinterventionen, Funktion der Aortenklappe und der Dimensionen der Aortenwurzel analysiert. Ergebnisse: Das mediane Alter bei der Operation betrug 6.3 Jahre (IQR: 1.4 – 13.6, 58% männlich), davon waren 10 Patienten in Säuglingsalter (20%). Innerhalb der medianen Beobachtungsdauer von 31.2 Monaten (IQR; 14.4-51) wurde bei frühem und spätem Todesereignis jeweils eines Patienten ein Gesamtüberleben von 95%, 5 Jahre nach der Operation beobachtet. Das Autograft musste bei zwei Patienten, 11 und 15 Monate nach initialer Ross-Operation ersetzt werden. Die Freiheiten von Reoperation am Autograft und am rechtventrikulären Ausflusstrakt waren 94% beziehungsweise 97%, nach 5 Jahren. Die Freiheit von einer mehr als milden Aortenklappeninsuffizienz betrug 97% nach 5 Jahren. Die Diameter der Aortenwurzel blieben bei der letzten Kontrolle auf allen Ebenen innerhalb der Normwerte stabil. Schlussfolgerung: Die modifizierte subkoronare Ross/Ross-Konno-Operation bei Kindern und jungen Erwachsenen zeigte exzellente früh- und mittelfristige Ergebnisse. Die vorgestellte Modifikation der Implantationstechnik des Autografts ist technisch reproduzierbar. Eine Ausweitung der Anwendung dieser im Erwachsenenalter validierten Technik auf das Kindesalter, könnte gerade in Hinblick auf die Erhaltung des Wachstumspotentials vorteilhaft sein

Psychosocial factors and their significance towards pain: a case study comparing monozygotic twins with AIS after spinal surgery

INTRODUCTION: Adolescent Idiopathic Scoliosis (AIS) is one of the most common spinal abnormalities in children, affecting 2% to 3% of adolescents in the United States. Its cause remains unclear. Many previous studies conclude that AIS may be caused by a combination of genetic and environmental factors, with very few consistencies. Severe scoliosis is usually treated with corrective surgery, and the etiology of post-surgical pain is even more unclear and has the opportunity to affect the patient well into adulthood. STUDY AIMS: By following a monozygotic twin pair with identical DNA, our retrospective case study can control for genetic disposition, and can look toward other possible causes for the pain the patients experienced. This study attempts to shed light on the complexities of AIS and pain with a focus on environmental and psychosocial factors. CASE PRESENTATION: We present a single pair of monozygotic twins treated for AIS with comparable spinal fusion surgeries performed at a large northeast urban children's hospital. Twin A and Twin B were initially treated with a brace for their scoliosis. Despite bracing, their curves progressed and warranted spinal fusion, with Twin A having a Cobb angle of 53°, and Twin B with 50°. The surgery was conducted simultaneously at the age of 13 by two different orthopedic surgeons. At age 7.5, Twin B was treated for Ebstein's anomaly of the tricuspid valve and significant dysrhythmias. METHODS: After the patients were discharged, a comprehensive retrospective chart review of the patients' pre-op, inpatient, and post-op pain and drug regiment was conducted. The patients were also asked to note their pain as they recovered after discharge. The patients and their mother completed self-report measures of multiple psychosocial variables both before and after surgery through REDCap. A Quantitative Sensory Test (QST) was also performed by the patients to assess their sensory sensitivity and pain thresholds. Mechanical, pressure, and thermal scores were obtained with the use of von Frey hairs, a pressure Algometer, and a Thermode. The QST was administered on the patients' palm/ thenar eminence (distant non-surgical site), and on their lower back (surgical site). The QST results were compared to a previous study's median cohort data, to discern if the patients presented hyper- or hyposensitivity for that particular test. RESULTS: Due to the limitations of case studies, the results presented here should be considered strictly preliminary. Twin B experienced more significant pain during both the acute and chronic recovery phases after surgery, and showed lower sensitivities during most pre-op QST trials. Twin B also scored markedly higher on a number of sub-variables in the psychosocial surveys. A notable correlation was the parent protective measure, indicating that the mother may have been more protective of Twin B. CONCLUSIONS: What is unique to this study is that age, gender, Cobb angle, fusion length, and genetic disposition are all controlled for, allowing us to analyze the patients based on other risk factors. Twin B shows consistently higher pain scores while in the hospital as well as while recovering at home. The parent self-report measures support these findings, showing a slight bias in favor of Twin B in regards to protectiveness, which also coincides with large-scale studies. Although preliminary, it is important not to underestimate the role environmental and psychosocial factors play in post-surgical pain

Genetics of Congenital Heart Malformations: Clinical and Molecular Studies

__Abstract__ Congenital heart malformations (CHM) are among the most common congenital defects, occurring in 8 out of 1000 live-births. In the past decade significant progress has been made in the identification of genes implicated in the signaling pathways involved in cardiovascular development. A major contribution has come from the study of model systems from fruit fly to mouse. Additionally, multiple disease genes implicated in genetic forms of human CHM have been identified, mainly through positional genetics in multiplex families. Especially human syndromes with CHM ("syndromic" CHM) have been instrumental in the elucidation of these disease genes, but recently also a number of disease genes implicated in "non-syndromic" CHM have been identified. In this chapter the most important forms of syndromic CHM with the different signaling pathways involved, and the genetic and environmental factors contributing to non-syndromic CHM are summarized. Several recent reviews have addressed the progress in the identification of these genes and pathways

Analysis of cardiac motion using MRI and nonrigid image registration

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Cardiac Arrhythmias

The most intimate mechanisms of cardiac arrhythmias are still quite unknown to scientists. Genetic studies on ionic alterations, the electrocardiographic features of cardiac rhythm and an arsenal of diagnostic tests have done more in the last five years than in all the history of cardiology. Similarly, therapy to prevent or cure such diseases is growing rapidly day by day. In this book the reader will be able to see with brighter light some of these intimate mechanisms of production, as well as cutting-edge therapies to date. Genetic studies, electrophysiological and electrocardiographyc features, ion channel alterations, heart diseases still unknown , and even the relationship between the psychic sphere and the heart have been exposed in this book. It deserves to be read