Dissecting the Management and Outcomes of Thoracic Aortovascular Disease During the COVID-19 Pandemic.

OBJECTIVE: The COVID-19 pandemic has forced the cancellation of planned surgery and led to significant surgical service reductions. Early intervention in aortovascular disease is often critical and cannot be deferred despite these reductions. There is urgent need to evaluate the provision and outcomes of thoracic aortovascular intervention during the peak of the pandemic. METHODS: Prospective data was collected for patients receiving open and endovascular thoracic aortovascular intervention over two-time points; January-May 2020 and January-May 2019 at three tertiary cardiovascular centres. Baseline demographics, cardiovascular risk and COVID-19 screening results were noted. Primary outcomes were median length of intensive care unit and hospital stay, intra-operative mortality, 30-day mortality, post-operative stroke, and spinal cord injury. RESULTS: Patients operated in 2020 (41) had significantly higher median EuroSCORE II than 2019 (53) (7.44 vs. 5.86, P = 0.032) and rates of previous cardiac (19.5% vs. 3.8%, P = 0.019), aortic (14.6% vs. 1.9%, P = 0.041), and endovascular (22.0% vs. 3.8%, P = 0.009) intervention. There was an increase in proportion of urgent cases in 2020 (31.7% vs. 18.9%). There were no intra-operative deaths in 2020 and 1 in 2019 (P = 1.00). There were no significant differences (P ≥ 0.05) in 30-day mortality (4.9% vs. 13.2%), median intensive care unit length of stay (72 vs. 70 hr), median hospital length of stay (8 vs. 9 days), post-operative stroke (3 vs. 6), or spinal cord injury (2 vs. 1) between 2020 and 2019 respectively. CONCLUSIONS: Despite the increased mortality risk of patients and urgency of cases during COVID-19, complicated by the introduction of cohorting and screening regimens, thoracic aortovascular intervention remained safe with comparable in outcomes to pre-COVID-19.

Duplicated left pulmonary artery: an unknown disease? Three case reports and review of the literature.

We report three cases of an abnormal finding of duplicated left pulmonary artery: two of these occurring in children with Kabuki syndrome and configuring the setting of a pseudo-pulmonary sling without any clinical or cardiac cross-sectional evidence of tracheal compression. The other case instead represents duplicated left pulmonary artery with pulmonary sling caused by the retro-tracheal course of the lower left pulmonary artery associated with "Christmas Tree" arrangement of the tracheo-bronchial system. In both patients with pseudo-pulmonary sling and Kabuki syndrome, the abnormal finding was incidental during echocardiographic examination and neither of the patients required surgical repair for the condition. To the best of our knowledge, they represent the third and fourth cases in which such an anomaly of the pulmonary artery branches not forming a sling is seen in association with Kabuki syndrome. Another case represents our second experience and the second case reported in literature with duplicated left pulmonary artery in the setting of a complex tracheal anatomy. In this symptomatic patient, surgical repair of atrial septal defect and relief of the vascular ring were indicated, and the surgical repair was performed successfully at the age of 3 years.

Options for the failing ventricle in pediatric heart disease.

The management of the pediatric patient with the failing ventricle poses its own therapeutic challenges, not least because patient size limits options available. Once medical management has hit its ceiling, attention is turned to surgical options for mechanical support. The approach to these options has to bear in mind that there may be many potential causes for pump failure, and that these occur often in the context of pulmonary hypertension and poor gas exchange. Although extracorporeal life support has been the mainstay of treatment for acute heart failure, in the last decade, attention has been focusing on longer-term options to bridge to recovery or eventual transplant. Added to this are more novel applications of ventricular assist devices, notable in the management of the failing Fontan circulation where there are no perfect solutions. There is growing interest in the use of such devices to power this delicate circulation and extend the functional capacity of patients without resorting to transplantation. In this review article, we explore the role each of these surgical modalities has to play in the management of the child with acute and chronic heart failure, and explore the recent developments in the rapidly growing field of pediatric ventricular assist.

Cardiac surgery outcome during the COVID-19 pandemic: a retrospective review of the early experience in nine UK centres.

BACKGROUND: Early studies conclude patients with Covid-19 have a high risk of death, but no studies specifically explore cardiac surgery outcome. We investigate UK cardiac surgery outcomes during the early phase of the Covid-19 pandemic. METHODS: This retrospective observational study included all adult patients undergoing cardiac surgery between 1st March and 30th April 2020 in nine UK centres. Data was obtained and linked locally from the National Institute for Cardiovascular Outcomes Research Adult Cardiac Surgery database, the Intensive Care National Audit and Research Centre database and local electronic systems. The anonymised datasets were analysed by the lead centre. Statistical analysis included descriptive statistics, propensity score matching (PSM), conditional logistic regression and hierarchical quantile regression. RESULTS: Of 755 included individuals, 53 (7.0%) had Covid-19. Comparing those with and without Covid-19, those with Covid-19 had increased mortality (24.5% v 3.5%, p < 0.0001) and longer post-operative stay (11 days v 6 days, p = 0.001), both of which remained significant after PSM. Patients with a pre-operative Covid-19 diagnosis recovered in a similar way to non-Covid-19 patients. However, those with a post-operative Covid-19 diagnosis remained in hospital for an additional 5 days (12 days v 7 days, p = 0.024) and had a considerably higher mortality rate compared to those with a pre-operative diagnosis (37.1% v 0.0%, p = 0.005). CONCLUSIONS: To mitigate against the risks of Covid-19, particularly the post-operative burden, robust and effective pre-surgery diagnosis protocols alongside effective strategies to maintain a Covid-19 free environment are needed. Dedicated cardiac surgery hubs could be valuable in achieving safe and continual delivery of cardiac surgery.

Aorto-Tracheopexy in the Treatment of Hunter Syndrome.

Hunter syndrome is a multisystem genetic disease in which a significant proportion of morbidity and mortality arise from respiratory dysfunction. Notably, tracheal abnormalities in Hunter syndrome can compromise clinical stability, and management is primarily supportive. We describe here the first successful implementation of aorto-tracheopexy in a 19-year-old patient as a surgical strategy to resolve progressive respiratory deterioration.

Long-segment tracheal stenosis: slide tracheoplasty and a multidisciplinary approach improve outcomes and reduce costs.

OBJECTIVE: Long-segment tracheal stenosis is rare, life-threatening, difficult, and expensive to treat. Management remains controversial. A multidisciplinary tracheal team was formed in 2000 to deal with a large number of children with airway problems referred for management. We review the effect of that service, comparing the era before and after the establishment of the multidisciplinary tracheal team. METHODS: From January 1998 through January 2004, 34 patients with long-segment tracheal stenosis (21 patients with cardiovascular anomalies) underwent surgical intervention. Cardiopulmonary bypass was used in all operations. Before the multidisciplinary tracheal team, pericardial patch tracheoplasty with or without an autograft technique was the preferred method of repair. After the multidisciplinary tracheal team, an integrated care plan preferring slide tracheoplasty was initiated, correcting cardiac lesions simultaneously. RESULTS: Before the establishment of the multidisciplinary tracheal team, pericardial patch tracheoplasty was performed in 15 of 19 patients. Twelve patients had a suspended pericardial patch tracheoplasty, 2 (17%) of whom died late after the operation. Of 3 patients who had had a simple unsuspended patch, 2 (67%) died early after the operation. Four patients were operated on with the tracheal autograft technique, 2 (50%) dying early in the postoperative period. After multidisciplinary tracheal team formation, in the era between 2001 and 2004, 15 patients were operated on with slide tracheoplasty, and there were 2 (13%) early postoperative deaths. A significant reduction in cost and duration of stay has been shown both in the intensive care unit and the hospital. CONCLUSION: Our data suggest that a formalized multidisciplinary team approach and a policy of primary slide tracheoplasty are beneficial in the management of children with long-segment tracheal stenosis.

Increasing donor-recipient weight mismatch in pediatric orthotopic heart transplantation does not adversely affect outcome.

OBJECTIVE: The aim of the study was to show the effect of heart transplant donor-recipient weight mismatch on mortality, right-ventricular (RV) failure, and medium-term control of systemic blood pressure. METHODS: From 2000 to 2008 inclusive, 161 patients undergoing orthotopic heart transplantation at our unit were retrospectively analyzed. The cohort was divided into three groups of similar size depending on the tertile ranges of the donor-recipient weight ratio. Median follow-up was 4.81 years. Donor-recipient body weight ratio was analyzed with respect to intubation time, time in intensive care unit (ITU), development of RV failure, medium-term survival, and freedom from medium-term hypertension. RESULTS: The median age was 115 months (23 days to 18 years), at a median weight of 26.9 kg (3-88 kg) at transplant. Median donor-recipient weight ratio was 1.61 (0.62-3.25). Mean intubation time was 448 h (SD 749.2), mean time in the ITU 302.7 h (SD 617.8). On linear regression, these were not related to donor-recipient weight ratio. A total of 38 patients (23.6%) developed postoperative RV failure. Nearly one-fifth (18.9) of patients in the lowest tertile group developed RV failure. In the middle tertile group, 24.5% developed RV failure and 28.8% in the upper tertile of weight mismatch, although this was not statistically significant (p = 0.48). On survival analysis, there was a higher mortality among those with the lowest tertile of mismatch (log-rank p = 0.04), but there was no difference in midterm survival on condition of survival to discharge (log-rank p = 0.14). There was also no association between weight ratio and freedom from medium-term hypertension as measured on serial 24-h ambulatory blood pressure monitoring (log-rank p = 0.39). There were nine patients in whom the weight mismatch was 3 or greater. There was no association between this 'extreme' mismatch group and either midterm mortality (p = 0.76) or freedom from hypertension (p = 0.62), but this was associated with the need for postoperative extracorporeal membrane oxygenator (ECMO) support (p < 0.01). CONCLUSIONS: Our current policy involves accepting a maximum donor-recipient weight ratio of 3. These encouraging findings cautiously justify this policy, in an era when marginal donors are increasingly sought.

Re-repair of the left atrioventricular valve in atrioventricular septal defects: the morphologic approach to the role of Gore-tex band reduction annuloplasty.

OBJECTIVES: Incompetence of the left atrioventricular valve remains the final Achilles' heel of repair of atrioventricular septal defects (AVSDs), despite low operative mortality in the modern era. We have analysed the morphological basis for valvar incompetence in our own series of repairs, and in doing so, reviewed the morphology of the annulus in AVSDs, before and after surgical repair. METHODS: We reviewed retrospectively re-repair operations of the left atrioventricular valve following previous repair of AVSDs at the Great Ormond Street Hospital from 1 January 1994 to 31 December 2007. From this case series, the reasons for valvar incompetence were identified and techniques used for re-repair recorded. We also incorporated the detailed morphology of the annulus of the left atrioventricular valve before and after repair from our cardiac morphology archive. RESULTS: Thirty-three patients had undergone re-repair of the left atrioventricular valve between 1 January 1994 and 31 December 2007. Twenty patients underwent re-repair of the left atrioventricular valve within 1 year of initial repair, and 13 cases beyond 1 year after repair. Cases re-repaired within 1 year mainly did so as a result of leaflet tears or valvar dysplasia. Cases re-repaired after this time mainly had multiple areas of valvar leakage, including central incompetence. Two patients underwent a second re-repair, which were dealt with by a partial ring from a thin-wall Gore-Tex graft for reduction annuloplasty. Out of the 121 cardiac morphologic specimens, 53 had undergone previous complete repair. Following repair, the annulus of the new left atrioventricular valve was composed of artificial patch material on its septal portion. CONCLUSIONS: Left atrioventricular valve incompetence following previous repair usually involves repair of the zone of apposition between the left bridging leaflets. Many of these valves also require annuloplasty to attain competence. Given the shape, growth potential and morphologic composition of the annulus in these cases, we have performed some of these repairs with a partial flexible ring from a thin-walled 3.5-mm Gore-Tex graft as a reduction annuloplasty that can be shaped to fit the exact contours of this annulus.

Mitral valve repair: critical analysis of the anatomy discussed.

In this brief review, we discuss the anatomy of the mitral valve pertinent to surgical repair. First, we emphasise the need for diagnosticians to describe the valve in the context of the position of the heart within the body, following the standard rules of anatomy, and using attitudinally appropriate descriptions. It has become customary to describe cardiac structures as if the heart is positioned on its apex. This cannot be good in the current era, when the tomographic techniques increasingly used for diagnosis demonstrate the heart as seen in the body. We then discuss the overall valvar structure in terms of a complex made up of the annulus, the leaflets, their tendinous cords, and the supporting papillary muscles. After providing accounts of the salient structure of each part of the complex, we discuss potentially divisive issues, such as the number of leaflets, and the categorisation of the tendinous cords. We explain how most of the disagreements stem not from differences in observation, but rather from differences in definitions. We suggest that these can largely be dissipated if the valve is analysed in its closed, rather than its open, position. When seen in the closed position, it becomes obvious that the key feature is the solitary zone of apposition between the major components of the skirt of leaflet tissue, this being the major functional part of the valvar complex. Finally, we discuss the relationships of the valvar complex to the other cardiac structures, concentrating on the other cardiac valves, the conduction tissues, and the coronary arteries and veins.

Late incompetence of the left atrioventricular valve after repair of atrioventricular septal defects: the morphologic perspective.

OBJECTIVE: The mortality following repair of atrioventricular septal defects has fallen dramatically in the last 4 decades, but reoperation for late regurgitation across the left atrioventricular valve has remained disconcertingly stagnant. Seeking potential structural causes, we compared the morphology of the surgically created septal leaflet of the left valve following repair of atrioventricular septal defects to the aortic leaflet of the normal mitral valve. METHODS: We compared the mitral valves of 92 normal hearts to the left ventricular components of the bridging leaflets of hearts with atrioventricular septal defect with common atrioventricular junction, determining the shape of the leaflets and the arrangement of the subvalvar apparatus. RESULTS: The aortic leaflet of the mitral valve is triangular compared with its rectangular septal counterpart after repair of atrioventricular septal defect. The cordal arrangement in the mitral valve is well organized, compared with the deficient cordal arrangement of the abnormal valve. A greater proportion of cords in the mitral valve divide to 3 generations (55.5% compared with 8.7%; P < .001), and a higher percentage of cords remain undivided in atrioventricular septal defects (60.8% compared with 25%; P < .001). CONCLUSIONS: Not only is the annular component in the left atrioventricular valve abnormal, but the subvalvar apparatus is characterized by deficiency and disarray. Furthermore, the axis of cordal insertion may potentiate to separation over the long term of the leaflets joined surgically. Valvar repair in this setting will never restore the arrangement of the normal mitral valve.

Development of the atrioventricular valves: clinicomorphological correlations.

The atrioventricular valves are formed from a complex arrangement of an annulus and leaflets, supported by a subvalvar apparatus that is composed of tendinous cords and papillary muscles. Although much has been said and written about their development, the exact nature of the process has yet to be fully elucidated. We believe that this is vital, since unraveling this complex process holds the key to the understanding of many of the congenital malformations that may afflict the valves.

Method and value of digital recording of operations for congenital heart disease.

PURPOSE: We describe the methods we have used to employ digital video techniques in the teaching of surgery for congenital heart disease. DESCRIPTION: This paper describes the hardware and software required to initiate and utilize digital video in surgery for congenital heart defects. Images are collected through a headlight camera and stored on standard digital-video camera tape. Tapes are catalogued using a purpose built database, linked to the hospital data system, and employing the European Pediatric Codes coding system for diagnostic and procedural coding. This permits keyword as well as patient-based searches. Master tapes are then digitally edited using proprietary software to create for example teaching tapes or to explain the operation to the family of the patient. EVALUATION: Eighty percent of the procedures performed by one surgeon (M.J.E.) during the last year have been recorded in this way. CONCLUSIONS: A working digital archive of pediatric cardiac surgical procedures has been created. Database links to hospital systems create the opportunity for linking full, visual records of procedures to be retained in an electronic patient record.

Problems with the right ventricular outflow tract: a review of morphologic features and current therapeutic options.

Repair of complex malformations that necessitate restoration of continuity between the right ventricle and the pulmonary arteries can now safely be performed with low morbidity and mortality. Major concerns still remain on the long-term outlook for these patients, and about the durability of the different prostheses used to restore that continuity, whether during initial correction or at the time of reintervention for failure of the conduit or pulmonary regurgitation. In this review, we discuss the salient morphologic features of the right ventricular outflow tract, and then focus on the indications for early and late intervention, current therapeutic options, and outcomes.