Coral reef aorta: a rare form of obstruction of the ascending aorta in adolescent with aortopathy- case report.

BACKGROUND: Supra aortic obstruction in children is uncommon and is seen in certain unique conditions. While intraluminal obstruction due to heavy calcification is seen in older populations, it is not described in pediatric populations. The coral reef aorta is a rare and distinct calcifying disease causing luminal obstruction of the suprarenal aorta in adults. The definition of this diagnosis relies entirely on the unique aspects and consistency of the lesions, which are rock-hard, irregular, gritty plaques with a white luminal surface resembling a coral reef. However, no such case has been described in children. CASE PRESENTATION: We present an adolescent boy who presented with a heavily calcified ascending aortic lesion associated with aortopathy and hypertension, 12 years after an aortic coarctation repair. The investigations included echocardiography, magnetic resonance and computer-tomographic imaging. A 3-D model was printed in order to visualize and plan surgical steps in advance for safe placement of clamps and defining the extent of resection. In addition, it provided an idea about tissue quality, thickness, spatial relationship, and orientation in relation to surrounding structures. Successful resection and replacement of the diseased segment of the aorta were achieved on cardiopulmonary bypass support. Post-operative recovery was uneventful, and at 6-month follow-up, the patient is doing well. In this report, various aspects of such lesions have been discussed, including clinical presentations, complications, planning and conduct of a safe cardiopulmonary bypass, and precautions during surgery for a successful outcome. CONCLUSION: Complicated obstructive aortic lesions in children require careful assessment, appropriate advanced imaging, and the use of 3-D printing technology in order to plan and perform safe and effective surgical management. The etiology of severe calcified aorta in children may be related to metabolic factors, previous surgery, use of a homograft, or an inflammatory process. However, it has yet to be proven.

Predictors of protein losing enteropathy after Fontan completion: An 8-year retrospective study at Sheikh Khalifa Medical City.

BACKGROUND: The Fontan procedure is the final stage of a three-stage palliation process in patients born with a univentricular heart as part of hypoplastic left heart syndrome (HLHS) or other pathologies with a univentricular heart. As essential as this procedure has proven to be for such cases, the Fontan physiology diminishes cardiac output and expands systemic venous pressure, which then leads to venous congestion that can be complicated by protein-losing enteropathy (PLE). This retrospective study aimed to identify the predictors of such complications in all patients who underwent completion of the Fontan procedure at our center (Sheikh Khalifa Medical City/SKMC) in the past eight years. METHODS: This study examined the medical records of patients who underwent completion of Fontan repair at our center since the inauguration of the cardiac surgery program of SKMC in the United Arab Emirates (UAE) - 01 Jan 2012 to 31 Dec 2020. Exclusion criteria included the absence of any of the undermentioned data in patient files. Patients were divided into two groups: those who developed PLE and those who did not. For each group, the following data were collected: demographics data (current age and age at completion of Fontan), clinical and laboratory data (oxygen saturation, serum albumin), echocardiographic data (classification of original cardiac diagnosis, degree of atrio-ventricular valve regurgitation, ventricular functions), hemodynamic data (mean pressures of superior vena cava and pulmonary arteries before Fontan completion), operative data (type of initial palliation, type of Fontan, presence of fenestrations and its size) and the need for any cardiac intervention prior to Fontan completion, such as atrio-ventricular valve repair, peripheral pulmonary stenting and arch balloon dilatation. RESULTS: Of the 48 included patients,13 (25%) developed PLE. Multivariate regression analysis proved that the best predictors of PLE were superior vena cava mean pressure (P = 0.012) and the degree of atrio-ventricular valve regurgitation (P = 0.013). An oxygen saturation <83% prior to Fontan completion was 92% sensitive in predicting PLE after Fontan completion. CONCLUSION: This is a single-center study of the predictors of PLE after Fontan procedure and, as expected from similar studies, SVC pressure higher than 11 mmHg and moderate-to-severe atrio-ventricular valve regurgitation were predictors of Fontan failure. The higher prevalence of PLE in our cohort, as well as lower cut-offs of SVC pressure that can predict complications, may be related to the predominance of hypoplastic left heart in the operated patients, which has been the main referral center for cardiac surgeries in UAE in the last decade.

Sternal Wound Management in Pediatric Cardiac Surgical Patients: A Novel Strategic Interprofessional Approach.

OBJECTIVE: Information on sternal wound management in children after cardiac surgery is limited. The authors formulated a pediatric sternal wound care schematic incorporating concepts of interprofessional wound care and the wound bed preparation paradigm including negative-pressure wound therapy and surgical techniques to expedite and streamline wound care in children. METHODS: Authors assessed knowledge about sternal wound care among nurses, surgeons, intensivists, and physicians in a pediatric cardiac surgical unit regarding the latest concepts such as wound bed preparation, NERDS and STONEES criteria for wound infection, and early use of negative-pressure wound therapy or surgery. Management pathways for superficial and deep sternal wounds and a wound progress chart were prepared and introduced in practice after education and training. RESULTS: The cardiac surgical unit team members demonstrated a lack of knowledge about the current concepts of wound care, although this improved after education. The newly proposed management pathway/algorithm for superficial and deep sternal wounds and a wound progress assessment chart were introduced into practice. Results in 16 observed patients were encouraging, leading to complete healing and no mortality. CONCLUSIONS: Managing pediatric sternal wounds after cardiac surgery can be streamlined by incorporating evidence-based current wound care concepts. In addition, the early introduction of advanced care techniques with appropriate surgical closure further improves outcomes. A management pathway for pediatric sternal wounds is beneficial.

The Sternal Wound Management in Pediatric Cardiac Surgical Patients: Implementation of Wound Care Pathways Incorporating Principles of Wound Bed Preparation Paradigm and Early-Advanced Therapy.

OBJECTIVE: The information on sternal wound management in children after cardiac surgery is limited. The authors formulated a pediatric sternal wound care schematic incorporating concepts of interprofessional wound care and the wound bed preparation paradigm including negative-pressure wound therapy (NPWT) and surgical techniques to expedite and streamline wound care in children. METHODS: Authors assessed knowledge about sternal wound care among nurses, surgeons, intensivists, and physicians in a pediatric cardiac surgical unit regarding the latest concepts such as wound bed preparation, NERDS and STONEES criteria for wound infection, and early use of NPWT or surgery. Management pathways for superficial and deep sternal wounds and a wound progress chart were prepared and introduced in practice after education and training. RESULTS: The cardiac surgical unit team members demonstrated a lack of knowledge about the current concepts of wound care, although this improved after education. The newly proposed management pathway/algorithm for superficial and deep sternal wounds and a wound progress assessment chart was introduced into practice. Results in 16 observed patients were encouraging, leading to complete healing and no mortality. CONCLUSIONS: Managing pediatric sternal wounds after cardiac surgery can be streamlined through incorporation of evidence-based current wound care concepts. In addition, the early introduction of advanced care techniques with appropriate surgical closure further improves outcomes. A management pathway for pediatric sternal wounds is beneficial.

Malposition of septum primum in isolated dextrocardia: unique and rare form of anomalous pulmonary venous return in association with partial absence of pericardium-case report.

BACKGROUND: Total anomalous pulmonary venous return (TAPVR) refers to an anomaly in which all of the pulmonary veins drain directly or indirectly to the systemic venous circulation. However, unusual types constitute approximately 5% or less of TAPVRs and there may be obstruction or discontinuity of pulmonary vein at various levels. CASE PRESENTATION: A 3-month-old infant was presented to us with history of poor feeding, respiratory distress and desaturations. The routine echocardiographic investigation initially confirmed the diagnosis of an atrial septal defect with dextrocardia. However, due to disproportionate severity of symptoms and congestive heart failure a cardiac computer tomography angiogram was done that revealed a rare finding of connection of pulmonary veins fused with the posterior atrium, but on the rightward side of the deviated atrial septum. Therefore, pulmonary veins entered a sinus that drains directly into the right atrial superior-posterior wall. During surgical repair, we found an area of absent pericardium in the diaphragmatic surface of the heart. The patient underwent total repair of the TAPVR and patch reconstruction of the pericardial defect. The patient is doing well at 6-month follow up. CONCLUSIONS: The septum primum malposition defect resulting in TAPVR is a very rare congenital anomaly that can be rarely seen without any heterotaxy. The anomalous features including absent pericardium and dextrocardia were present in this patient have not been described previously with TAPVR. Therefore, we have hypothesized the embryological correlation of absent pericardium and cardiac malposition in such case. Transthoracic echocardiography with Doppler interrogation is a reliable method for diagnosing this condition. In case of suboptimal echocardiographic image due to cardiac position, unclear anatomy or unexplained symptoms, advanced imaging such as computer tomographic angiography or cardiac magnetic resonance imaging can be very helpful. Preoperative proper diagnosis of this anomaly facilitates successful surgical management with excellent outcome.

Long-term follow-up after recanalisation of aortic arch atresia.

AIMS: Aortic arch atresia (AAA) is one of the rarest obstructive defects. The presence of this anomaly in adult age is uncommon. The typical anatomic feature consists of a complete occlusion of the membranous obstruction resulting in an acquired atresia without flow continuity between the proximal and distal segments. This feature is important in determining the feasibility of percutaneous intervention. The aim of the present study was to share long-term follow-up data of adult patients with AAA requiring percutaneous interventions for the management of this rare anomaly involving five different centres. METHODS AND RESULTS: Retrospective data of 19 patients (12 males, 63.2%, mean age 32.2±18.9 years) diagnosed with AAA treated in five different centres between 1999 and 2017 were collected. All patients underwent percutaneous recanalisation by (1) radiofrequency (RF) system (five patients, 26.3%), (2) extra-stiff guidewire (12 patients, 63.2%), and (3) transseptal needle (two patients, 10.5%). All procedures were subsequently followed by covered stent implantation. Two patients developed complications during the procedure and one of them died. Over a median follow-up of 4.94 years, four (21%) patients were able to be weaned from medications for hypertension. All the patients underwent reassessment for recurrence or restenosis during the follow-up. Seven (36.8%) patients underwent successful stent dilatation with a balloon. After the intervention, one patient experienced a late complication; however, one patient died due to an unknown cause believed to be unrelated to the previous recanalisation procedure. CONCLUSIONS: Percutaneous treatment of AAA is feasible with good long-term survival. This study reports the largest case series so far available in the literature.

Congenital aneurysmal right coronary artery with a fistula to the left atrium in an adult.

BACKGROUND: Congenital coronary artery fistula in association with aneurysm of the involved coronary artery in adults is rare. Moreover, the right coronary artery- left atrial fistula is also uncommon. Most of the cases are asymptomatic. However, symptomatic patients need therapeutic interventions. The potential complications associated with this anomaly are life-threatening, therefore, there is a need to explore more on differential diagnosis, investigations, management strategies and prevention of complications. CASE PRESENTATION: We present herewith a 26-year-old male patient with symptoms of chest pain and dyspnea. He was diagnosed with aneurysmal dilatation of the right coronary artery in its entire course which terminated as a fistulous communication into the left atrium. The closure of the fistula was done using autologous pericardial patch under cardiopulmonary bypass. Currently, the patient is being followed up after surgery and receiving anticoagulants. CONCLUSION: The advancement in the diagnostic imaging modalities have made it possible to find similar abnormalities more frequently. Due to rare nature of this anomaly, there is a need to explore and discuss management strategies that include medical management, surgical intervention or percutaneous interventions for a successful outcome.

Stent dilatation of atretic aortic coarctation in an adult-case report and literature review.

BACKGROUND: Patients with functional aortic interruption of the descending thoracic aorta at the isthmus due to severe coarctation in association with atretic lumen are extremely rare in the adult population. The management is challenging and carries high morbidity and mortality. CASE PRESENTATION: We describe successful percutaneous reconstruction using a covered stent in a similar patient who is doing well two-years after intervention. A literature search was done to explore management strategies and their long-term outcomes for better understanding. CONCLUSIONS: This report is an attempt to highlight the role of minimal invasive approach in the management of rare, severe coarctation of the aorta in adult patients to avoid morbidity and mortality associated with more invasive procedures.

Three-dimensional printed prototypes refine the anatomy of post-modified Norwood-1 complex aortic arch obstruction and allow presurgical simulation of the repair.

Three-dimensional (3D) printed prototypes of malformed hearts have been used for education, communication, presurgical planning and simulation. We present a case of a 5-month old infant with complex obstruction at the neoaortic to transverse arch and descending aortic junction following the neonatal modified Norwood-1 procedure for hypoplastic left heart syndrome. Digital 3D models were created from a routine 64-slice CT dataset; then life-size solid and magnified hollow models were printed with a 3D printer. The solid model provided further insights into details of the anatomy, whereas the surgical approach and steps of the operation were simulated on the hollow model. Intraoperative assessment confirmed the anatomical accuracy of the 3D models. The operation was performed in accordance with preoperative simulation: sliding autologous flaps achieved relief of the obstruction without additional patching. Knowledge gained from the models fundamentally contributed to successful outcome and improved patient safety. This case study presents an effective use of 3D models in exploring complex spatial relationship at the aortic arch and in simulation-based planning of the operative procedure.

Large cardiac fibroma and teratoma in children- case reports.

Primary cardiac tumours in paediatric population are an unusual occurrence. Although, majority of such tumours are benign (90%), the frequency and type of cardiac tumours in this age group is different from the adult population. There are several consecutive series published in the last decade on cardiac neoplasms. Therefore, this is not only an effort to contribute to the existing literature for better understanding and management of similar patients but also to highlight the importance of early detection either by prenatal imaging or careful evaluation of differential diagnosis of common symptoms. We herein, describe two infants with large cardiac tumours (fibroma and teratoma) both arising from the interventricular septum and underwent surgical excision. A possible role of cardiac remodeling in myocardial tissue healing after extensive tissue resection in such patients is hypothesised through available experimental or limited clinical information.

Primary lymphoepithelioma-like carcinoma of lung.

A 57-year-old Caucasian woman presented with nonproductive cough. Computed tomography revealed a peripheral solid mass in the upper lobe of the left lung. She underwent thoracotomy and upper lobectomy. Histology of the excised tumor demonstrated lymphoepithelioma-like carcinoma of the lung, with no associated Epstein-Barr virus activity. Being a rare entity and mostly seen in Asians, very few cases have been described previously.