Successful Late Repair of Truncus Arteriosus: A Single Center Experience.

Background: Truncus arteriosus is a rare congenital heart defect resulting from the failure of the truncus arteriosus to divide during fetal development. It leads to a single outflow tract from the heart and, if left untreated, can be fatal. Late presentation and repair can also increase the risk of pulmonary hypertensive crises, which can lead to morbidity and mortality after repair. Methods: We performed a retrospective study examining outcomes of late-presenting patients who were repaired for this anomaly at our institution. Results: We identified seven patients who underwent late repair of truncus arteriosus who were 3 to 11 years of age. There were six females and one male. Postoperatively, all patients showed improvement in symptoms and hemodynamic parameters, with no reported mortality. The median duration of stay in the intensive care unit was nine days and with a range from 3 to 18 days, while the median hospital stay was 29 days with a range from 21 to 60 days. Conclusion: These findings highlight the potential for successful outcomes even in cases of delayed diagnosis.

Structural aortic wall abnormalities following the Nikaidoh operation, which could be reversible and include a healing process.

The Nikaidoh operation continues to be used for patients with transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction. We recently reported structural and functional changes in the aortic root during the follow-up of a patient who underwent the Nikaidoh operation. These changes necessitated re-operation. The pathophysiology of these changes and their potential for reversibility have not yet been studied. In this communication, we describe the extensive structural changes in the aortic wall of the same patient.

Novel concepts and early results of repairing common arterial trunk.

OBJECTIVES: Common Arterial Trunk (CAT) continues to have a very poor prognosis globally. To address that, we have developed a novel technique targeting key concepts for the correction of all components of the anomaly, using autologous arterial tissue. This aims to enhance results, availability worldwide, and importantly to avoid the need for repeated reoperations. METHODS: From January 2019 to 4 January 2021, all patients with isolated CAT had repair of the defect using autologous arterial trunk tissue with direct right ventricle (RV) to pulmonary artery (PA) connection. Clinical outcomes, follow-up which included multi-slice computed tomography 3D segmentation and 4D cardiovascular magnetic resonance flow, are presented. RESULTS: Twenty patients were included in the study (median age 4.5 months). There were 2 hospital deaths due to systemic infection and pulmonary hypertensive crisis, respectively. Following discharge all patients remained asymptomatic with no signs of heart failure and improved pattern of growth (median follow-up: 8 months). Early postoperative 3D segmentation showed a conical shaped neo-right ventricular outflow chamber connecting the body of the RV to the main PA through a valveless ostium, and normal crossing of PA and neo-aorta. 4D cardiovascular magnetic resonance pattern of flow showed normal rapid laminar flow through the atrioventricular valves followed by a vortex towards the outflow tracts. There was laminar flow through the neo-aorta and neo-PA with velocity not exceeding 2.5 m/s. The PA regurgitant fraction was 25 ± 5% and was limited to early diastole. CONCLUSIONS: The initial results of utilizing the key concepts, using autologous arterial tissue for the repair of CAT, are encouraging, both clinically and by multimodality imaging.

Characterization of morphology and function of the 'neo-atria' after a modified Mustard operation.

Background: There is a pressing need to improve early and long-term results of the Mustard operation. A modification of the operation was introduced at the Aswan Heart Centre for this purpose which relies on creating new functional atria rather than the two rigid channels in the classical Mustard operation. Objectives: To evaluate the morphology and function of the neo-atria, shortly after modified mustard operation for a 'neglected' patient with TGA, VSD and severe pulmonary hypertension. Methods: A 6-year-old with neglected TGA, VSD and pulmonary hypertension presented with severe cyanosis, clubbing and haemoconcentration (Hb 22 g/dL), underwent the modified Aswan-Mustard operation (MAM) with rapid smooth postoperative recovery. Repeated 2D echograms and multi-slice CT scans, followed by 3D segmentation, were performed after the operation. The size, shape, and morphology of the neo-atria were measured and measurements of the patterns of instantaneous filling and emptying of the right and left ventricles were quantified. Results: The neo-systemic venous atrium consisted of three components with a combined volume of 78 mL/m2, all of which contributed to the reservoir, conduit, and importantly contractile function of the neo-atrium. The pulmonary venous atrium consisted of two components with a combined volume of 66 mL/m2. These measurements were made at atrial end diastole. The volumes of the systemic venous and the pulmonary venous diminished to 51 and 54 mL/m2, respectively, at the end atrial systole - indicating relatively preserved contractile functions. Conclusion: Following the modified Aswan-Mustard operation, neo-atrial function was relatively well preserved compared to the classical operation. The long-term results of these findings and their effects on quality of life need to be studied further.

Three-dimensional images of a pulmonary dominant truncus arteriosus before and after a novel repair.

This paper documents, for the first time, the in vivo size, geometry, and function of the different components of this important subtype of truncus arteriosus (pulmonary dominant). Previous descriptions were based on examining formalin-fixed (collapsed) specimens, or descriptions during operations. It is hoped that this information can be of value in designing operative treatment as well as interpreting future sequential imaging, with the aim of optimizing the results of comprehensive repair.

A New Technique for Shaping the Aortic Sinuses and Conserving Dynamism in the Remodeling Operation.

BACKGROUND: Preserving dynamism and recreating the sinuses in the Dacron graft are thought to be important for optimizing results of aortic valve-conserving operations. METHODS: We describe a novel technique that preserves dynamism and recreates the sinotubular junction. In addition, it tailors 3 sinuses of defined longitudinal and transverse curvatures in a straight Dacron tube during the operation. The technique has been used in 6 patients with varied aortic root pathology. We performed preoperative and postoperative multimodality imaging using computerized image analysis as well as 3-dimensional models. RESULTS: There was no early or midterm death. Upon discharge, patients were clinically well, with echocardiographic evidence of minimal (3 patients) or mild (3 patients) aortic regurgitation. Computed tomography and cardiac magnetic resonance imaging with extensive image analysis of the aortic root size, shape, and function showed partial or complete normalization of these parameters. This included the shape and dynamism of the aortic annulus and the size and shape of the geometric (effective) orifice. The 4-dimensional magnetic resonance imaging pattern of flow in the sinuses and ascending aorta showed favorable vortices in the sinuses, right-handed helical flow, and marked diminution of energy loss in the ascending aorta. CONCLUSIONS: The novel technique described here is simple, practical, and cost-effective because it uses a widely available straight Dacron tube. The technique does not use rigid internal or external support. The early results are encouraging. Larger series with longer follow-up are required.

Management of a huge right atrial thrombus in a patient with multiple comorbidities.

BACKGROUND: Floating right heart thrombi (RHT) represent an underdiagnosed, potentially hazardous, and to some extent rare phenomenon in patients presenting with acute pulmonary embolism (APE). Emergent treatment is usually required for such a condition. CASE PRESENTATION: A 19-year-old young lady presented with progressive shortness of breath, marked renal impairment, thrombocytopenia, and a highly oscillating huge right atrial mass. After she was admitted to the intensive care unit, she arrested in asystole and was resuscitated, and her electrocardiogram (ECG) showed evidence of acute anterior myocardial infarction. Urgent cardiac surgery to remove the right atrial mass was proposed by the heart team as the best option of management. Surgery was emergently performed with extra-corporeal membrane oxygenator (ECMO) as a support. Following surgery, mechanical support and vasopressors were successfully weaned and the patient achieved a good recovery. CONCLUSIONS: A pulmonary embolism response team (PERT) approach should always be considered where a multidisciplinary team involving a cardiologist, radiologist, cardio-thoracic surgeon, radiologist, and intensivist shall determine the management strategy for a challenging presentation of a massive pulmonary embolism or floating right heart thrombi causing the hemodynamically unstable clinical condition.

Thrombosis of mechanical mitral valve prosthesis during pregnancy: An ongoing "saga" in need of comprehensive solutions.

Emergency treatment for thrombosed mechanical valve prothesis during pregnancy is not uncommon in low- and middle-income countries. The presence of a mechanical valve continues to be an important cause of maternal morbidity and mortality. There is a pressing need for increasing awareness and feasible solutions for this huge problem. We here describe four patients who needed emergency treatment for thrombosis of mechanical valve prothesis during pregnancy and review the evolving comprehensive strategies for dealing with this issue.

Pulmonary artery banding in a modified Mustard operation improves biventricular geometry and function.

Patients with transposition of great arteries, with intact interventricular septum (TGA-IVS) and deconditioned left ventricle, represent a considerable challenge in developing countries. The modified Mustard operation was shown to provide a significant improvement for these patients, particularly by enhancing atrial functions and left ventricular filling. Yet, the problems of the systemic right ventricular dysfunction and the resulting secondary tricuspid regurgitation (TR) remain to be of major concern. In addition, the deviation of the interventricular septum towards the left side markedly impairs ventriculo-ventricular interaction and predisposes to dynamic left ventricular outflow tract obstruction (LVOTO). We report that adding a moderately loose pulmonary artery banding to the modified Mustard operation in a case of TGA-IVS results in improvement of biventricular geometry and function, tricuspid and mitral valve functions and disappearance of dynamic LVOTO.

Revival and modification of the Mustard operation.

OBJECTIVE: The neonatal arterial switch operation is currently the procedure of choice for patients with transposition of the great arteries. However, a large number of patients present too late for the arterial switch operation and are best managed with the atrial switch operation. METHODS: We have used the Mustard operation in its original form or following a new modification designed to enhance the atrial functions and filling of the left ventricle in an attempt to improve long-term results. RESULTS: Between July 2013 and November 2018, a total of 101 patients underwent the Mustard operation, 86 with the new modification. The median age at operation was 16 months (6 months to 27 years). A total of 75 patients (74.3%) were male. Median preoperative oxygen saturation was 71%. There were no early deaths and there were 3 late deaths during a median follow-up period of 24.2 months (all in patients with large ventricular septal defect and established pulmonary vascular disease). At the latest follow-up, all patients were in stable sinus rhythm. There were no baffle leaks. Seven patients had asymptomatic narrowing of the superior baffle, and 1 patient required balloon dilatation. Follow-up is 100% complete and includes computed tomography and magnetic resonance imaging at regular intervals (75 patients to date). Computerized analysis of representative subsets showed enhanced rate and pattern of filling of the left ventricle in the modified operation compared with the classic operation. CONCLUSIONS: The use of the Mustard operation, particularly the modified technique should play an important role in treating late-presenting patients with transposition of the great arteries. Improving the pattern of filling of the left ventricle could enhance the long-term results of the Mustard operation.

Characterization of size, shape and pattern of flow in the neo-aorta and pulmonary artery in a patient following an innovative technique of repair for truncus arteriosus.

Background. Truncus arteriosus (TA) caries a very poor prognosis. In the absence of early correction, only 12 percent of patients born with this anomaly survive beyond one year. There is no agreement about the best method of surgical correction of this anomaly. We have devised an innovative valveless technique using autologous arterial tissue to repair TA. Objectives. Characterizing the size, shape and pattern of flow in the neo-aorta and pulmonary artery, in a patient following the new technique. Patient and Methods. Cardiac MRI and multislice CT imaging, followed by offline computerized image analysis was used in a patient aged 3 months, within 3 weeks of operating. Results. The size, shape and topology of the neo-aorta and pulmonary artery, approximated that present in normal hearts. The pattern of flow in the reconstructed vessels was laminar, throughout the cardiac cycle with minor acceleration during systole. The pulmonary regurgitation resulting from the absence of a valve occurred during late diastole, and was well tolerated. The size of the right ventricle diminished considerably following operation, and the right ventricular ejection fraction was supernormal. Conclusion. This early study in one patient provides new unique data of the size, shape, topology and pattern of flow in the neo-aorta and pulmonary artery, which appear to approximate normality. The long-term results of this promising operation need to be studied.

Rheumatic aortic valve disease-when and who to repair?

Rheumatic valve disease is an endemic problem that is responsible for substantial morbidity and mortality in many countries. Unlike the rheumatic mitral valve, aortic repair continues to be challenging. A thorough understanding of the underlying mechanisms; structural and functional, is essential for repair. We here describe various methods of repair and outline our favoured techniques.

NOBLE and EXCEL: The debate for excellence in dealing with left main stenosis.

Left main coronary artery (LMCA) disease is associated with increased morbidity and mortality. Coronary artery bypass grafting surgery (CABG) has always been the standard revascularization strategy for this group of patients. However, with the recent developments in stents design and medical therapy over the past decade, several trials have been designed to evaluate the safety and efficacy of percutaneous coronary intervention (PCI) as an alternative to CABG surgery in patients with LMCA disease. Recently, the results of two major trials, EXCEL and NOBLE, comparing CABG versus PCI in this patient population have been released. In fact, the results of both trials might appear contradictory at first glance. While the EXCEL trial showed that PCI was non-inferior to CABG surgery, the NOBLE trial suggested that CABG surgery is a better option. In the following review, we will discuss some of the similarities and contrasts between these two trials and conclude with lessons to be learned to our daily practice.

Three-dimensional transesophageal echocardiography incremental value in a rare case of a bileaflet tricuspid valve.

Detailed assessment of the tricuspid valve using two-dimensional echocardiography is always challenging, as only two of three leaflets can be seen at a time. Three-dimensional echocardiography can provide the enface view of the tricuspid valve that allows simultaneous visualization of all of the three leaflets. In a 42-year-old male patient scheduled for pulmonary endarterectomy, 3DTEE showed that the tricuspid valve is bileaflet, with one septal and another lateral leaflet. There were two commissures, one of them is anteriorly positioned and the other one is posterior. Our findings were confirmed intra-operatively by direct surgical visualization of the tricuspid valve.

Repair of very severe tricuspid regurgitation following detachment of the tricuspid valve.

We report on the case of 5-year-old girl with severe tricuspid regurgitation following previous repair of double outlet right ventricle with subaortic ventricular septal defect, performed through trans-atrial approach using detachment of tricuspid valve leaflet. The severe tricuspid regurgitation was found to be due to dehiscence at the site of the previous detachment and was repaired using a pericardial patch. In this report, we discuss the relative merits and risks of using this technique.