Influence of Aortic Arch Morphology on Renal Perfusion in Patients with Coarctation of the Aorta: An Exploratory Study.

Objectives: The configuration of the aortic arch, particularly a Gothic arch shape, in individuals with corrected coarctation of the aorta (CoA) has been associated with a decreased systolic wave amplitude across the arch, which could potentially impair renal perfusion and elevate the risk of arterial hypertension. This study aims to explore the relationship between the morphological characteristics of the aortic arch and their impact on renal perfusion in patients with CoA. Methods: Seventy-one subjects with corrected CoA underwent continuous 24 h ambulatory blood pressure monitoring, computed tomography to assess the aortic arch, and renal perfusion scanning. Subjects were stratified into three groups based on the height-to-width (H/W) ratio of their aortic arch: Group 1 with a H/W ratio of <0.65, Group 2 with a H/W ratio between 0.65 and 0.85, and Group 3 with a H/W ratio of >0.85. Results: Groups 1 and 2 (53,78% and 62.63%) presented with a higher hypertension prevalence of elevated blood pressure than Group 3 (38.89%). Notable variations were observed among the subjects in the time to peak perfusion (Tmax) in the left kidney across the groups. Group 1 showed a median Tmax at 0.27, Group 2 at 0.13, and Group 3 at -0.38 (p-value = 0.079). The differences in Tmax for the right kidney followed a similar trend but were not statistically significant (Group 1 at 0.61, Group 2 at 0.22, and Group 3 at 0.11; p-value = 0.229). Conclusions: This study suggests that variations in the aortic arch morphology might not significantly influence renal perfusion in CoA patients. This indicates the potential adaptability of the renal blood flow, which appears to compensate for reduced perfusion, thus minimizing adverse effects on the kidney function. This adaptability suggests an inherent physiological resilience, emphasizing the need for further targeted research to understand the specific interactions and impacts on treatment strategies for CoA.

Giant aortic aneurysm repair in a child due to arterial tortuosity syndrome.

Arterial tortuosity syndrome is an extremely rare hereditary connective tissue disorder. We present a case of an incidentally diagnosed aneurysm of the aortic root and the ascending aorta caused by arterial tortuosity syndrome, which was confirmed genetically. The aneurysm was repaired surgically. One year after the procedure, there was no further dilation of the aorta or formation of new aneurysms.

Influence of the COVID-19 pandemic on the congenital heart surgery service in Lithuania.

INTRODUCTION: CHD is a unique group of medical pathologies. Literature worldwide reports significant decrements in the case volume of patients with these conditions due to the recent global pandemic of coronavirus disease 2019. The only centre providing congenital cardiac care for Lithuanian population is in a hospital which was the main medical institution for the sickest coronavirus disease 2019 patients. Hence, this centre had to maintain its service alongside the mobilisation of resources to tackle the crisis. AIM OF STUDY: To evaluate the effect of the pandemic on the service of congenital heart surgery in Lithuania. METHODS: The activity of a single centre providing congenital heart care working in a main coronavirus 2019 pandemic hospital during the pandemic was analysed and compared to a matched period of pre-pandemic activity. RESULTS: The number of admitted patients was similar during both pre-pandemic and pandemic periods. During the pandemic period, younger patients were more often operated as urgent cases. Their postoperative length of stay was longer. However, there were no differences in early postoperative mortality between the two groups. CONCLUSIONS: It was possible to maintain an accessible and high-quality specialised congenital cardiac care for various age patients during global pandemic events, while working in the main pandemic hospital.

Early and Late Outcomes after Arterial Switch Operation: A 40-Year Journey in a Single Low Case Volume Center.

Background and Objectives: The results of the arterial switch operation in large congenital heart centers are excellent, and the results in small and medium centers are improving. The objective of this article is to share our experience utilizing the international knowledge transfer program to improve early and late arterial switch operation outcomes in our center. Materials and Methods: A retrospective analysis of patients who underwent the arterial switch operation in Vilnius University Santaros Clinics Cardiothoracic Surgery Center between 1977-2020 was performed. Results: A total of 127 consecutive arterial switch operations were performed in our center. Surgical mortality during the entire study period was 24.6%. Surgical mortality prior to the program, during the program, and after the program was 88.24%, 41.7%, and 5.81%, respectively (p < 0.0001). The surgical mortality of patients operated on during the last 10 years was 4%. The overall survival estimate for the 97 surviving patients was 96.9%, 94.9%, 93.8%, 93.8%, 93.8%, 93.8% at 1, 3, 5, 10, 15, and 20 years, respectively. Risk factors for early mortality included longer aortic cross-clamp time and operation prior to the knowledge transfer program. The only significant risk factor for late reintervention was concomitant aortic arch obstruction treated at the time of the arterial switch. Conclusions: The surgical treatment of transposition of the great arteries by means of an arterial switch with good results can be possible in low-to-medium volume congenital heart surgery centers. International knowledge transfer programs between high-expertise high-volume congenital heart centers and low-to-medium volume congenital heart centers may help to shorten the learning curve and improve early and late outcomes after an arterial switch. The risk factors for surgical mortality and intervention-free survival in low-volume surgical centers are similar to those in high-volume centers. Late arterial switch-related complications are similar to those among different-sized congenital heart centers.

Interventions after Arterial Switch: A Single Low Case-Volume Center Experience.

Background and Objectives: With the growing population of arterial switch operation survivors, the rate of late complications associated with the operation is growing as well. The aim of this publication is to share our experience and encourage collaboration between congenital cardiac surgeons and interventional cardiologists in treating late complications after arterial switch operation. Materials and Methods: A retrospective analysis of Vilnius University Santaros Clinics Cardiothoracic Surgery Centre arterial switch operation survivors who underwent additional treatment for late neo-pulmonary artery stenosis and aortic arch obstruction between 1989 and 2019 was conducted. Results: Out of 95 arterial switch operation survivors 14 (15%) underwent 36 reinterventions. The majority were treated for neo-pulmonary stenosis. The median time from arterial switch operation to the first reintervention was 1.4 years (interquartile range, 2 months to 2.4 years). 1, 3, 5, and 10 years intervention-free survival in patients treated for neo-pulmonary stenosis and aortic arch obstruction was 98, 94, 94, and 93% vs. 95, 94, 94, and 93%, respectively. There were no complications associated with redo surgical procedures, while eight patients who underwent catheter-based interventional treatment had treatment-related complications, including one death. Conclusions: Both neo-pulmonary stenosis and aortic arch obstruction (new aortic coarctations or aortic recoarctations) tend to develop in the first decade after an arterial switch operation. Surgical and catheter-based interventional treatment with good results is possible even in a small volume center. Close collaboration of the congenital heart team (congenital cardiac surgeons and interventional cardiologists) in choosing the best treatment option for an individual patient helps to minimize the risk of potential complications.

Prevalence of arterial hypertension, hemodynamic phenotypes, and left ventricular hypertrophy in children after coarctation repair: a multicenter cross-sectional study.

BACKGROUND: This study aimed to evaluate hemodynamic phenotypes and prevalence of left ventricular hypertrophy in children after coarctation repair with right arm and leg blood pressure difference < 20 mmHg. Secondary objectives were analysis of effects of age at intervention, residual gradient across the descending aorta, and type of correction. METHODS: Blood pressure status and left ventricular hypertrophy were diagnosed according to European Society of Hypertension 2016 guidelines. RESULTS: Of 90 patients with a median age 12.5 (8.9-15.8) years, 8.5 (6.0-11.8) years after coarctation repair who were included, 42 (46.7%) were hypertensive. Isolated systolic hypertension dominated among 29 hypertensive patients with uncontrolled or masked hypertension (25 of 29; 86.2%). Of the 48 patients with office normotension, 14.6% (7) had masked hypertension, 8.3% (4) had ambulatory prehypertension, and 54.2% (26) were truly normotensive. Left ventricular hypertrophy was diagnosed in 29 patients (32.2%), including 14 of 42 (33.3%) hypertensive and 15 of 48 (31.3%) normotensive patients. The peak systolic gradient across the descending aorta was greater in hypertensive subjects (33.3 ± 12.7 mmHg) compared with normotensive subjects (25 ± 8.2 mmHg, p = 0.0008). Surgical correction was performed earlier than percutaneous intervention (p < 0.0001) and dominated in 40 of 48 (83.3%) normotensive versus 24 of 42 (57.1%) hypertensive patients (p = 0.006). CONCLUSIONS: Arterial hypertension with isolated systolic hypertension as the dominant phenotype and left ventricular hypertrophy are prevalent even after successful coarctation repair. Coarctation correction from the age of 9 years and older was associated with a higher prevalence of hypertension.

Surgical Treatment of Infective Endocarditis in Pulmonary Position-15 Years Single Centre Experience.

Background and Objectives: Infective endocarditis in the pulmonary position is a rare disease. Isolated pulmonary valve endocarditis is extremely rare. The aim of our study was to assess patients who were treated surgically for pulmonary endocarditis at our institution from January 2003 to December 2017. Materials and Methods: We analyze eight cases of infectious endocarditis in pulmonary position out of 293 patients who were operated for infective endocarditis (2.7%, 8/293). Only two of these eight patients were not related to congenital heart malformation. They were followed for early and late mortality, long-term survival, postoperative morbidity and reoperations. Results: Among six patients suffering from congenital heart disease, four patients underwent corrections of pulmonary valve malformation previously, and their infected grafts were replaced by two allografts and two xenografts. The two other patients had replaced their infected pulmonary valves with allografts. Two non-congenital patients with pulmonary valve endocarditis underwent valve replacement with biological prosthesis. All patients survived the early postoperative course. The mean follow-up time was 9.1 (interquartile range (IQR), 5.3-12.6) years. The long-term follow-up included seven patients. One patient (12.5%, 1/8) died more than 4 years after the surgery due to sepsis. Pulmonary endocarditis was the rarest endocarditis treated surgically (p < 0.001). Conclusion: Surgery for infective endocarditis in the pulmonary position (IEPP) is an effective method of treatment with excellent early outcome and good late results despite a very uncommon pathology and few operations being performed. Surgery performed earlier may make the procedure less radical.

30-year experience of Fontan surgery: single-centre's data.

BACKGROUND: The Fontan procedure has been modified several times since it was introduced into practice in 1968. As many patients now survive to adulthood, attention is directed towards their clinical status and late morbidity. We report our surgical experience of 30 years in Fontan procedures. METHODS: From January 1985 to January 2015, 80 patients underwent Fontan surgery. Twenty-one patients received an atrio-pulmonary Fontan (Group I), four patients underwent total cavopulmonary connection (TCPC) with an intra-atrial lateral tunnel (Group II), six patients received extra-cardiac TCPC with an aortic homograft (group III) and 49 patients received extra-cardiac TCPC with an expanded polytetrafluoroethylene conduit. They were followed for early and late mortality, long-term survival, postoperative morbidity and reoperations. RESULTS: The mean follow-up time was 7.4 ± 6.6 years. The Kaplan-Meier estimated 15-year survival rate was 42% in Group I, 50% in Group II, 83% in Group III and 94% in Group IV. The median length of stay in intensive care unit, intubation and chest drain stay time were 90 h (IQR, 46-119), 8 h (IQR, 6-16) and 18 days (IQR, 12-28) respectively. Early complications were bleeding (6), taken down of Fontan circulation (3) and acute heart failure managed by left heart bypass (1). Late-occurring morbidities included arrhythmias (6), protein-losing enteropathy (2), thromboembolism (2) and tracheal stenosis (1). Fourteen patients (18%) had redo Fontan procedures. CONCLUSION: Our series showed improving results after Fontan completion with excellent mid-term outcome after extra-cardiac TCPC with expanded polytetrafluoroethylene conduit. The long-term result should be followed.

Primary cardiac lymphoma: two cases and a review of literature.

BACKGROUND: Primary cardiac lymphoma is one of the rarest tumours of the heart. It belongs to the extra-nodal non-Hodgkin's lymphomas. The most common type of this tumour is diffuse large B cell lymphoma. Usually, right atrium and right ventricle are involved. This tumour is fatal unless diagnosed and treated in time. In this article two female patients who were diagnosed with primary cardiac lymphoma and treated at our clinic are described. The first patient went to remission after the treatment, while the second patient died. The goals of this article are to show the difficulties of diagnosing and treating this disease, the role of cardiac surgery in its treatment and to raise awareness of this disease. CASE REPORTS: In this article two female patients who were diagnosed with primary cardiac lymphoma and treated at our clinic are described. The first patient went to remission after the treatment, while the second patient died. CONCLUSIONS: Primary cardiac lymphoma is a very rare disease. At the moment the most effective treatment is chemotherapy. Palliative surgery may be necessary to correct hemodynamics when venous blood flow to the lungs is disturbed.

Fontan Completion for Younger than 3 Years of Age: Outcome in Patients with Functional Single Ventricle.

Most single ventricle patients undergo Fontan procedure earlier in current era. However, optimal timing of Fontan completion after an intermediate staging surgery is controversial. We present children who had Fontan surgeries younger than 3 years of age comparing the data with older patients. Between 2000 and 2013, 45 patients with functional single ventricle underwent extracardiac total cavopulmonary connection (TCPC) using GORE-TEX(®) conduit at a single institution. Children were divided into group A (age < 36 months; n = 15) and group B (age ≥ 36 months; n = 30), and retrospectively reviewed. Median follow-up was 2.9 [interquartile range (IQR) 2-4.4] years. There were two hospital deaths and one late death (all in group B, p = 0.286). One TCPC was taken down in group A. The median intensive care unit and hospital stay were 90 (IQR 46-140) h and 21 (IQR 16-33) days for group A versus 65 (IQR 45-115) h and 29 (IQR 20.8-38.5) days for group B, respectively (p = 0.322 and p = 0.057). Ventricular ejection fraction and GORE-TEX(®) conduit size were similar in both groups. The time of chest drain stay was significantly lower in group A (median 12 days, IQR 7-22) than in group B (22 days, IQR 16-32.5) (p = 0.014). Extracardiac TCPC can be performed earlier with good intermediate results. Earlier Fontan procedure might be advantageous for reducing chest drains stay.

Surgical repair of a giant congenital right atrial aneurysm: a case report.

Giant congenital right atrial aneurysms are rare defects of the heart. Though usually asymptomatic, they can be potentially life-threatening. Major risks include heart failure, supraventricular arrhythmias, rupture of the wall of the aneurysm. This defect is usually diagnosed incidentally. It is commonly found when transthoracic echocardiography or chest X-ray is performed. In some cases computed tomography or cardiac magnetic resonance imaging is needed to establish the diagnosis. Potential therapeutic options include surgery, catheter ablation and conservative follow-up. Currently preferred method for correcting this defect is surgical excision of the aneurysm, when surgical indications are met. In this article we describe a successful aneurysmectomy performed on a 16-month old female infant, who at the time of hospitalization presented with severe heart failure and symptoms of cardiac tamponade.

Surgical correction of atrial septal defect in the elderly.

INTRODUCTION: Atrial septal defects (ASDs) are one of the most common congenital malformations in adults. Correction of ASDs in advanced age remains controversial, even though beneficial effects in this patient group were found in recent studies. In older patients, less invasive transcatheter closure of ASDs has been recommended. AIM: The aim of this study was to analyze our advanced age ASD surgical cohort: early and late results. MATERIAL AND METHODS: Retrospective analysis of 32 patients operated on at an age of ≥ 60 years (i.e. age 66.13 ± 4.8, range from 60 to 78) in our center between 2001 and 2011 was carried out. We reviewed our experience of surgical ASD closure in elderly patients over a 10-year period to assess the effects of this type of treatment on early postoperative and long-term survival, early and late complications, preoperative and postoperative clinical status (New York Heart Association [NYHA] functional class), pulmonary hypertension (PH) and atrial arrhythmias. The patients were divided into two groups according to age. RESULTS: The frequency of comorbidities was lower in younger age group patients (11 [61%] vs. 13 [93%], p < 0.05). Atrial fibrillation/flutter was found in 21 (66%) of all patients. Late postoperative mortality was higher in the older patient group (3 [21%] vs. 1 [5.6%]). Despite this, we observed significant improvement of symptoms and functional ability in the older population after surgical ASD closure (group I, n = 10 [56%] vs. group II, n = 12 [86%]). CONCLUSIONS: Surgical correction of clinically significant ASD is effective even in older patients with comorbidities.

Sustainable knowledge transfer in pediatric cardiac surgery: a team approach to shared learning.

The study assesses the impact of a program using a model of knowledge transfer on the long-term development of a pediatric cardiac service in Lithuania. A team from the United Kingdom evaluated Lithuanian pediatric cardiac services and provided support in areas targeted for improvement. The total number of infant operations performed in Lithuania from 1990 to 2008, together with in-hospital mortality rates, was broken down into 3 time periods and analyzed to estimate the efficacy of the program: (1) 1990-1998, before the program; (2) 1999-2002, during the program; (3) 2003-2008, following the end of the program. Lithuanian results in children older than 1 year were comparable with other European centers. However, only 315 infant and neonatal cardiac procedures were performed between 1990 and 1998, and there was an in-hospital mortality of 34.2%. Between 1999 and 2002, the UK team performed 23 highly complex demonstration procedures (in-hospital mortality, 13%). During the same period the Lithuanian team performed 305 additional operations in neonates and infants, and in-hospital mortality decreased to 18.7%. From 2003 to 2008 results continued to improve-559 infant operations were performed, with in-hospital mortality of 11.3% (P < .0001). Knowledge transfer has led to substantial and sustainable long-term improvement in the results of infant cardiac surgery in Lithuania. Demonstrating techniques and care on a limited number of more complex cases is an efficient way of transferring knowledge and skills to the developing pediatric cardiac centers.

CT angiography of mildly symptomatic, isolated, unilateral right pulmonary vein atresia.

We report a mildly symptomatic 12-year-old boy with a very rare congenital anomaly-isolated unilateral pulmonary vein atresia. Diagnosis was made using CT angiography and its role in diagnosis is discussed.

[Surgical treatment of critical aorta coarctation in neonates]. / Naujagimiu aortos koarktaciju chirurginis gydymas.

UNLABELLED: Coarctation of the aorta is relatively common defect accounting for 5-8% of all congenital heart defects. Newborns very often, about in 50% of cases, are in cardiogenic shock. From 1991 to 2003, 26 neonates with coarctation of the aorta underwent surgical repair at Heart Surgery Center of Vilnius University: 12 with isolated coarctation of the aorta and 14 with leading heart pathology. Age of patients was from 2 to 30 days. Weight was from 2.8 to 4.7 kg. Fifty percent of neonates were on ventilatory and inotropic support. All neonates were operated using surgical technique "end-to-end" or extended "end-to-end" in case of aortic arch hypoplasia. All neonates with isolated coarctation of the aorta survived; with leading heart pathology mortality was 14.3%. From 26 operated neonates 2 had recoarctation, 1 - reoperation and 2 - balloon aortoplasty. Our patients were free from spinal chord ischemia and brain damage. CONCLUSIONS: Neonatal coarctation of the aorta needs an urgent surgical treatment. Surgery remains a gold standard for the treatment of aorta coarctation. Preferable technique is "end-to-end" anastomosis or extended coarctation resection with "end-to-end" anastomosis for hypoplastic aortic arch. Associated cardiac pathology increases risk of operation.

[Congenital heart surgery in neonates]. / Naujagimiu igimtu sirdies ydu chirurgija.

From January 1974 trough August 2002 184 neonates with congenital heart disease underwent surgical repair in Heart Surgery Clinic of Vilnius University. It came to 3.8% of all 4813 procedures of congenital heart diseases performed. Number of neonates operations considerably increased in the latter's years, because special Department with proper technique was established. One hundred and seven neonates were operated without cardiopulmonary bypass with 36.4% postoperative mortality rate. Seventy seven neonates underwent surgery repair with cardiopulmonary bypass. Postoperative mortality rate was 67.5% in this group. Mean age of patients was 11.96+/-0.28 days. Unsatisfactory results of neonates operations with cardiopulmonary bypass, especially such as left heart hypoplasia syndrome is the main problems. The group of specialists performing repair of congenital heart diseases is working actively in the heart Surgery Clinic of Vilnius University. Improvement of surgery methods and results, following the best Heart Surgery Clinics of the world, is the main purpose of this group.

[Closure of atrial septal defect via small lateral thoracotomies, partial and full length sternotomies]. / Priesirdziu pertvaros defekto pasalinimas per nedideles sonines torakotomijas, daline bei pilnutine sternotomijas.

Surgical closure of atrial septal defect is considered a low risk procedure. Various minimally invasive surgical techniques spread over the conventional full-length sternotomy. But the risk profile and complication rates of the minimally invasive closure of atrial septal defect are not revealed sufficiently. We present the results of atrial septal defect surgery via small thoracotomies: right anterolateral, right posterolateral, partial sternotomies and full-length median sternotomies. Ninety four patients underwent surgical closure of atrial septal defect via minimal approaches through the period from February 1999 to December 2001. Via median sternotomies were operated 85 patients. Right anterolateral group consisted of 17 patients, right posterolateral - 68, partial sternotomies - 9. The intracardiac period of operation was performed on electrical fibrillation under mild hypothermia. Median bypass time was 33 min. in right anterolateral, 31 min. - right posterolateral, 32 min. - partial sternotomies, 37 min. - median sternotomies. Median fibrillation time was 21 min. in right anterolateral, 18 min. - right posterolateral, 15 min. - partial sternotomies, 21 min. - median sternotomies. There were no technical procedure related complications in minimally invasive group. Intrahospital morbidity consisted of 6 pneumonias, 4 pleural effusions. There were 3 pneumonias, 2 pleural and 2 pericardial effusions in median sternotomies group.