Long-term outcomes of the classic Konno-Rastan procedure in paediatric and adult patients: impact of aortic annulus size on patient outcomes.

OBJECTIVES: The classic Konno-Rastan procedure may yield different outcomes regarding aortic annulus diameters ≤15 mm and larger. Focusing on the effect of the diameter of the aortic annulus, we described the long-term outcomes of our patients. METHODS: The outcomes of paediatric and adult patients who underwent surgery from 2000 to 2021 were studied retrospectively. The patient population was divided into 2 groups with aortic annulus diameters ≤15 mm and >15, and the outcomes were compared between the 2 groups. RESULTS: A total of 48 patients, with a mean age of 12.24 ± 9.42 years (2-53 years) and a median follow-up duration of 8 years (7 months to 20 years) with an IQR of 5.5, were enrolled. The mean peak instantaneous pressure gradient was 78.97 ± 25.29 mmHg, which decreased to 21.43 mmHg (P-value = 0.012). The maximum left ventricular outflow tract gradient at the last follow-up was 28.21 mmHg, with the exception of 1 case with a gradient of 68.45 mmHg. The mean diameter of the aortic annulus was 15.34 ± 3.87 mm (8-23 mm), and the mean prosthetic valve size was 20.31 mm, which was 5 mm (33%) larger than the native annulus diameter. The overall mortality rate was 6.3%, with 1 death in the hospital and 2 in the first year after the surgery. The major complication rate, including mortality, heart block and reintervention, was higher in patients with ≤15 mm annulus (P-value = 0.028.) However, there was no difference between the 2 groups in follow-up. Four (8%) late cardiac reoperations were performed, none of which were related to our surgeries. CONCLUSIONS: Kono-Rastan surgery for patients with aortic valve diameter of ≤15 mm can be performed with acceptable long-term outcomes.

Minimally invasive versus sternotomy approach for double-patch repair of partial anomalous pulmonary venous connection and sinus venosus defect in pediatric and adult patients: Mid to long-term outcomes.

BACKGROUND: Several surgical techniques have been proposed to repair right partial anomalous pulmonary venous connection (PAPVC) along with sinus venosus defect (SVD). This study aimed to compare the perioperative data and outcomes of double-patch repair using a minimally invasive approach versus conventional sternotomy in pediatric and adult patients. METHODS: This retrospective study was conducted on 48 minimally invasive cases and 35 sternotomy cases, undergoing surgery by a single surgeon between July 2002 and August 2020. For all patients, repair was performed using the double-patch technique. In the minimally invasive approach, right mini-thoracotomy was performed with central cannulation for children and with peripheral cannulation for adults. The patients were classified into two pediatric and adult groups, and each group was categorized into minimally invasive and sternotomy approaches. They were followed-up by transthoracic echocardiography and electrocardiography before and early after surgery, 3 and 6 months after surgery, and then annually. The relative data were compared between the two approaches in terms of perioperative findings, postoperative pulmonary vein or superior vena cava (SVC) stenosis, and sinus node dysfunction. RESULTS: This study included 25 minimally invasive cases and 19 sternotomy cases in the pediatric group (mean age, 4.99 ± 4.28 and 6.10 ± 4.39 years, respectively) and 23 minimally invasive cases and 16 sternotomy cases in the adult group (mean age, 35.73 ± 8.06 and 32.62 ± 9.80 years, respectively). The mean and median follow-ups were 6.31 ± 4.92 years and 6 years (range: 6 month-18 year) in the pediatric group and 6.15 ± 4.53 years and 5 years (range: 6 month-18 year) in the adult group, respectively. The mean chest tube drainage was significantly lower in the minimally invasive pediatric group (p = .03), and the mean blood transfusion volume was significantly lower in the minimally invasive adult group compared to the other groups (p = .03). No stenosis occurred in the pulmonary veins. Mild SVC stenosis occurred in one patient in the minimally invasive pediatric group, with no need for reintervention. All patients had a normal sinus rhythm, except for the mentioned case with a transient, first-degree atrioventricular block, which spontaneously reverted to the normal sinus rhythm. CONCLUSIONS: The minimally invasive approach can be a safe and practical alternative for the double-patch repair of PAPVC and SVD. It ensures a repair with comparable quality to sternotomy, but with better cosmetic and psychological outcomes.

Ascending aorta graft pseudoaneurysm and aortobronchial fistula caused by a fractured sternal wire: a case report.

BACKGROUND: Pseudoaneurysm of ascending aorta is a rare but serious complication of cardiovascular surgeries and it infrequently occurs in the normal prosthetic graft materials. We share our experience with an unusual case of ascending aorta Dacron graft pseudoaneurysm caused by a fractured sternal wire. CASE PRESENTATION: A 34-year-old man, known case of Marfan syndrome, with history of two prior aortic surgeries for aneurysm of ascending aorta, arch and thoracoabdominal aorta, presented with hemoptysis. The hemoptysis originated from an aortobronchial fistula secondary to a huge ascending aorta Dacron graft pseudoaneurysm. The graft erosion and subsequent pseudoaneurysm was caused by a fractured sternal wire. Surgical repair of the pseudoaneurysm was performed successfully and a Gore-tex patch was placed behind the sternum over the graft to prevent further direct contact of the wire and the graft. CONCLUSION: Sternal wires can damage the adjacent vascular grafts and lead to fatal complications such as pseudoaneurysm formation. Thus, preventive measures such as using sternal bands and placing a covering layer between the sternal wires and aortic grafts are recommended in patients with dilated or replaced ascending aorta.

Huge Coronary Aneurysm in a Morbidly Obese Man with Exertional Dyspnea and Chest Pain.

BACKGROUND Giant coronary artery aneurysm (GCAA) is a rare disease, with an incidence of 0.02% in the general population. GCAA is defined as when the diameter of the coronary artery is more than 4 times the adjacent part or more than 8 mm. There are several causes of GCAA, with atherosclerosis being the most common. Patients with giant coronary artery aneurysms can be asymptomatic or develop chest pain, dyspnea, and palpitations. Complications of GCCA include myocardial infarction, thrombosis, and sudden death, so early treatment is necessary to prevent mortality. There is no standard surgical approach for a giant coronary artery aneurysm. CASE REPORT A 64-year-old man with hypertension, opium addiction, morbid obesity (body weight 151 kg and BMI 46), and benign prostate hyperplasia presented with a giant coronary aneurysm in coronary angiography. The patient underwent cardiac surgery, and a 42-mm coronary aneurysm was detected. The aneurysm had many orifices that opened to the left main coronary artery, left circumflex artery, LAD, the diagonal branch of the LAD, and the septal branch of the LAD. Aneurysmectomy and coronary artery bypass graft were successfully performed. CONCLUSIONS Giant coronary artery aneurysms are rare. Patients with giant coronary artery aneurysms may experience sudden death due to myocardial infarction and other cardiovascular complications due to ischemia. Because it is rare, there is no standard surgical approach for a giant coronary artery aneurysm. Further studies need to focus on standardized surgical management of patients with giant coronary artery aneurysms.

Midterm follow up of transcatheter closure of coronary artery fistula with Nit-Occlud® patent ductus arteriosus coil.

BACKGROUND: Coronary artery fistula (CAF) is a rare congenital anomaly with a challenging scenario in children. This study reports our experience in transcatheter closure of CAF with Nit-Occlude PDA coil and midterm clinical and imaging follow-up. METHODS: Twelve children with congenital CAF between 2009 and 2019, mean age 2.05 ± 2.05 years (4 days to 7.2 years), mean weight 8.8 ± 4.83 (2.8-17 kg), who underwent transcatheter closure with PFM coil at the Namazi hospital, Shiraz, Iran, were reported. Echocardiography and electrocardiogram were done before and after the procedure (early, 3, and 6 months after), and Multi-slice computerized tomography or conventional coronary angiography was performed at least one year after closure. RESULTS: In a median follow-up of 5.5 years (range 13 months to 8 years), retrogradely closed fistula had no residual, and the fistula tract was wholly occluded, but in most anterogradely closed fistula, had a small residual, which made the fistula tract open and need additional coil closure. CONCLUSIONS: Transcatheter closure of CAF with PFM coil is feasible and effective with low mortality and morbidity, although antegrade closure with this device may be accompanied by residual shunt and need for multiple coil insertion.

Using the autologous innominate vein as a substitute for pulmonary arteries in a patient with pulmonary atresia and absent pulmonary arteries.

BACKGROUND: Repair of the absence of the whole or major parts of pulmonary arteries is a challenge, and the choice of conduit material to reconstruct the pulmonary arteries is under dispute. We used the autologous innominate vein to construct pulmonary arteries. CASE PRESENTATION L: We present a novel technique using the autologous innominate vein as a free graft in a 6-month-old infant with pulmonary atresia and absence of central pulmonary arteries. Double ductus arteriosus were the only source of perfusion of the lungs. The innominate vein was substituted for the central pulmonary artery between the two lung hila. Total repair by using Contegra graft was performed 9 months later. The patient has been followed for 5 years. CONCLUSIONS: The autologous innominate vein could be used as inter-hilar pulmonary arteries with no calcification and fibrosis in 5-year follow-up.

A Woman with Systemic Lupus Erythematosus and Odd Valvular Presentation: A Case Report.

BACKGROUND Systemic lupus erythematosus (SLE) is a systemic disease with various cardiac and non-cardiac presentations. We present the case of a young woman with odd presentation of SLE mistakenly identified as a valve abscess that was scheduled for surgery. CASE REPORT This 35-year-old woman presented with rapid progression of aortic stenosis, and the transesophageal echocardiography report showed a misdiagnosed aortic web (congenital) and aortic wall abscess. She was scheduled for surgery as a case of subacute bacterial endocarditis (SBE) and aortic abscess, despite lack of fever. CONCLUSIONS Cardiovascular involvement should be considered in any SLE patient, especially those with high SLE scores, even with negative antiphospholipid antibody. Cardiovascular involvement may be odd and misleading in some cases, which may warrant especial attention and experienced caregivers for clinical reasoning and proper management.

Investigation of associated factors with post-operative outcomes in patients undergoing Tetralogy of Fallot correction.

BACKGROUND: Tetralogy of Fallot (TOF) is one of the congenital cardiac abnormality which occurs during embryonic time. Although surgical correction, especially early operation, is the best way to treat patients, still contributing factors in morbidity and mortality is controversial. The objective of this study is to investigate potential factors which might be correlated with post-operative outcomes of TOF. METHODS: In this retrospective study, 349 monitored patients with TOF correction were selected. Median of age was 4 (0.66-8) year, 58% of patients were male and 42% were female. Time of inotropic drug, extubation time, and ICU stay were considered as post-operative outcomes which extension of each of them increased the risk of death. RESULTS: Ventricular septal defect enlargement was associated with longer extubation time and ICU stay. Higher ratio of pre-operative haematocrit was correlated with mortality (0.047). CONCLUSIONS: Results of this study demonstrated that haematocrit ratio before operation should be considered as a predictive factor, and patients with higher ratio of haematocrit require more care after operation. VSD enlargement is associated with longer extubation time and ICU stay.

Use of Cardiopulmonary Bypass for Management of Massive Air Embolism During Hysteroscopic Metroplasty.

The patient was a 32-year-old woman who presented with infertility secondary to uterine didelphys. Hysteroscopic metroplasty was chosen as the corrective surgical procedure for this anatomical defect. During the surgical repair, the patient developed a massive air embolism (MAE) leading to hypotension, arrhythmia, and cardiogenic shock. Resuscitation was started by placing the patient in the right-side up position, and emplacement of central venous catheter, but it was unsuccessful. The decision was then made to bypass the patient's cardiopulmonary system to effectively treat the MAE. Cannulation was done via femoral vein and artery. During cardiopulmonary bypass (CPB), the MAE was quickly eliminated, oxygen saturation was normalized, and the patient was hemodynamically stabilized. The surgical repair was successfully completed and the patient was decannulated and recovered without any incident.

Noninvasive Assessment of Pulmonary Artery Pressure in Patients with Extracardiac Conduit Total Cavopulmonary Connection.

To assess quantitative measurement of mean pulmonary artery pressure (PAP) in extracardiac total cavopulmonary connection (TCPC) patients by noninvasive echocardiographic inferior vena cava collapsibility index (IVC-CI) and also correlation between the peripheral vein pressure and mean PAP. In 19 TCPC patients with at least 1-year follow-up after completion of TCPC, complete echocardiography including IVC-CI was recorded. All patients underwent cardiac catheterization for mean PAP pressure, peripheral vein pressure (PVP) and contrast study. Different cutoff points of mean PAP were analyzed, and based on the highest cutoff point, patients were categorized into two groups: mean PAP < 17 mmHg (acceptable) and > = 17 mmHg (high) for comparison analysis. Between 2006 and 2015, from 43 patients who had undergone TCPC, nineteen patients with a mean age 12.9 ± 6.6 years and mean follow-up time of 3.4 ± 1.9 years were included in this study. Mean PAP was 14.6 ± 3.97 (range 8-22 mmHg). Thirteen (68 %) patients had PAP < 17 mmHg. No significant statistical difference was detected with respect to age, sex, type of congenital heart disease and fenestration between these two groups. But, right ventricular dominancy was more prevalent in the high PAP group (50 % vs. 7.7 % P value 0.03). IVC-CI had a correlation with mean PAP (r 0.67, P < 0.001). IVC-CI < 21.8 % can predict PAP > = 17 mmHg with 83 % sensitivity and 100 % specificity. Regression analysis proposed an equation for PAP measurement: PAP (mmHg) = 20.2097-0.1796 × (IVC-CI), (r2 = 0.56). Peripheral vein pressure measurement also showed a good correlation with mean PAP and may be used to estimate PAP with the following equation: PAP (mmHg) = 0. 8675 × PVP, (r 0.90, P < 0.0001). In conclusion, IVC-CI as noninvasive and peripheral vein pressure measurement as a minimal invasive method may be useful for quantitative estimation of PAP in patients with extracardiac TCPC.

Transcatheter Closure of Partially Ligated Vertical Vein after Surgical Correction of Supracardiac Total Anomalous Pulmonary Venous Connection.

Total anomalous pulmonary venous connection (TAPVC) is an anomaly in which the pulmonary veins are directly connected to one of the systemic veins or drain into the right atrium. Management of pulmonary hypertension after the total correction of this congenital cardiac anomaly is very important. Unligation of the vertical vein in the supracardiac type of this anomaly can be a draining pathway for the prevention of postoperative pulmonary hypertension crisis. Late onset transcatheter closure of the unligated vertical vein after a decrease in pulmonary pressure with the Amplatzer vascular plug type 1can prevent residual left-to-right shunting. Here we describe two patients who previously underwent surgical correction of supracardiac TAPVC and their vertical veinwas partially ligated due to severe pulmonary hypertension. Consequently, because of increased left-to-right shunting in the follow-up period, transcatheter occlusion of the vertical vein was done for them and this procedure seemed safe and less invasive compared to the surgical approach.At 2 years'follow-up, there was marked pulmonary artery pressure and clinical improvement.

Persistent Truncus Arteriosus With Intact Ventricular Septum: Clinical, Hemodynamic and Short-term Surgical Outcome.

INTRODUCTION: Truncus arteriosus with intact ventricular septum is a rare and unique variant of persistent truncus arteriosus (PTA) which usually presents with central cyanosis and congestive heart failure in neonate and early infancy. Associated cardiac and non-cardiac anomalies may affect morbidity and mortality of these patients. CASE PRESENTATION: We describe clinical presentation, echocardiography and angiographic features of a 7-month old boy with PTA and intact ventricular septum who underwent surgical repair of the anomaly at our institution. Operative findings, surgical procedure and short-term outcome are reported. CONCLUSIONS: While our patient had systemic pulmonary arterial pressure at the time of complete surgical repair, it was improved after surgery.

Evaluation of electrocardiographic changes after arterial switch operation.

BACKGROUND: Transposition of Great Arteries (TGA) is a serious congenital heart disease which can be accompanied by good outcomes with anatomic correction within the first few weeks of life. OBJECTIVES: The present study aimed to evaluate electrocardiographic changes in the children with uncomplicated Arterial Switch Operation (ASO). PATIENTS AND METHODS: Twelve lead electrocardiograms were obtained from thirty-three patients with TGA after ASO. Then, the patients' QT-dispersion and P-wave dispersion were compared to those of 33 age- and gender-matched individuals as the normal control group. Both groups were also evaluated by M-mode echocardiography. Student's t-test and Pearson correlation were used to analyze the data. Besides, P < 0.05 was considered as statistically significant. RESULTS: The mean age of the patients and the control group was 41 ± 3.7 and 40.12 ± 4.2 months, respectively. Comparison of P wave, T wave, QRS complex, PR interval, QT segment, and corrected QT segment showed significant differences in the frequency of inverted T wave in pericardial leads [V3, V4, V5, and V6] (P = 0.004; more in patients), P wave amplitude in lead II (P < 0.001; more in patients), R wave amplitude in V1 (P = 0.016; smaller in patients), R and S waves amplitude in V6 (P = 0.004 and P = 0.001; taller in patients), corrected QT segment (in lead V5; P < 0.0001: prolonger in patients), and PR interval (P = 0.001; prolonger in patients). However, no significant differences were found regarding the vector axis and heart rate. Right bundle branch block (18% vs. 0%), Bifascicular (3% vs. 0%), and first-degree blocks (15% vs. 0%) were significantly more in the patients. Besides, the PR interval was longer in the corrected complex TGA (146 ± 24.4 vs. 127.7 ± 23.1, P = 0.001). Moreover, R/S ratio in lead V1 was significantly prolonger, among the patients (2.86 ± 3.35 vs. 0.706 ± 0.53, P = 0.002). Nonetheless, no significant was observed between the patients and controls concerning the mean of QT dispersion. On the other hand, the two groups were significantly difference in terms of P wave dispersion (25.7 ± 13.8 vs. 33.74 ± 12.9, P = 0.024). CONCLUSIONS: In this study, first-degree block and right bundle branch were detected in the operated patients with TGA. Increased P dispersion in these patients may increase the risk of atrial arrhythmia. Thus, long-term follow-up is necessary in these patients.

Complete heart block following transcatheter closure of atrial septal defect due to growth of inflammatory tissue.

Transcatheter closure of atrial septal defect (ASD) is the most common approach to close the defects worldwide. In this approach, persistent conduction disturbance is extremely rare, but an acute increase in supraventricular ectopy and minimal risk of atrioventricular conduction disturbance, as complete heart block, has been seen. Here, we report a patient who underwent ASD closure with device and presented with persistent complete heart block 10 days after device occlusion due to inflammatory tissue formation just near the atrioventricular node area at the floor of the right atrium without any direct compression on the triangle of Koch.

Evaluation of noninvasive methods for the diagnosis of fungal endocarditis.

Fungal endocarditis (FE) is an uncommon disease with a high risk of morbidity and mortality. Here, we evaluated the different methods for diagnosing this infection. Cardiac valve, vegetation, and embolic materials obtained during surgery were examined for fungal infections by direct smear and culture. At least two blood samples were inoculated at the bedside into BACTEC medium. Galactomannan, mannan Ag enzyme-linked immunosorbent assay, and real-time polymerase chain reaction (PCR) assay were performed with serum samples. Of 25 patients with suspected infective endocarditis (IE), 8 were found to have proven FE according to the direct culture results. The etiologic agents were Aspergillus niger (three cases), A. flavus (two cases), A. fumigatus (one case), and Candida albicans (two cases). Blood culture was positive in only 1 case. The sensitivity, specificity, positive and negative predictive values, and positive and negative likelihood ratios of the results from the galactomannan test were 83.3%, 84.2%, 62.5%, 94.1%, 5.3, and 0.2; these same values, obtained from real-time PCR, were 87.5%, 94.4%, 87.5%, 94.4%, 15.6, and 0.14, respectively. Because mannan antigen was positive in samples from only one patient, we opted not to calculate the sensitivity. However, the specificity value in 23 cases without IE caused by Candida spp. was 100%. Based on our results, both the galactomannan test and real-time PCR can serve as reliable, noninvasive tests for the diagnosis of FE, compared with culture, which is considered to be the gold standard.

Evaluation of thymic changes after median sternotomy in children.

In patients who undergo median sternotomy to treat congenital heart diseases, a thymectomy is performed to yield better access to the cardiac system. In this study we have used MRI to evaluate the changes in size, shape and location of the thymus after midsternatomy. This case-control study was performed during 2011-2012 in Shiraz, Iran. Eligible participants between 5-17 years of age were divided into case and control groups (n=13 per group). Each participant underwent a median sternotomy at least one year prior to study entry. Participants were initially examined by a cardiologist and then referred for MRI. A radiologist examined all MRI images. The thymus was observed in all control group patients and in only 7 (53.8%) patients in the case group. There was a significant relationship noted in terms of mean age in the group whose thymus was visible and the group in which the thymus was not visible. We have observed no significant difference in thymic visibility between these two groups based on the mean age at midsternatomy. In pediatric patients undergoing cardiac surgery the possibility of remaining or regenerated thymic tissues may be evaluated by MRI. The remaining portion of the thymus may have any shape, size or location. Therefore, it can be misinterpreted as a mass if a patient's previous surgical history and age at the time of surgery are not taken into consideration.

Incidence and outcome of documented fungal endocarditis.

BACKGROUND: Fungal endocarditis, the most severe form of infective endocarditis, is characterized by excessive mortality and morbidity. OBJECTIVES: The present study aimed to analyze the characteristics of fungal endocarditis to improve the management of these patients. MATERIALS AND METHODS: In this cross-sectional study, vegetations on the mitral or tricuspid valves and embolic material surgically removed from the patients with suspected infective endocarditis between December 2009 and November 2011 were examined for fungal infection by direct smear and culture, and the susceptibility patterns of the isolated species were determined. Then, blood samples were cultured on BACTEC media and real-time PCR was done with blood and tissue samples. RESULTS: Of the 31 patients with suspected infective endocarditis who did not respond to antibacterial therapy, 11 had confirmed fungal endocarditis. The most frequent predisposing risk factors were previous surgery and drug abuse. The organisms isolated were Aspergillus spp. and Candida albicans. Resistance to amphotericin B and itraconazole was observed in Aspergillus species, and to fluconazole in Candida albicans. Positive PCR results were obtained in blood and tissue samples. CONCLUSIONS: Fungal endocarditis should be considered in the patients not responsive to antimicrobials. Moreover, management of these patients can be improved with molecular diagnostic methods and by determining the susceptibility patterns of the etiologic agents.

Evaluation of cardiac and valvular function after arterial switch operation: a midterm follow-up.

OBJECTIVES: Transposition of Great Arteries (TGA) is a serious congenital heart disease and anatomic correction in the first few weeks of life has revealed good outcomes nowadays. In this study, we aimed to evaluate the myocardial and valvular function at midterm postoperative follow-up. PATIENTS AND METHODS: In this study, thirty-three patients with TGA and Arterial Switch Operation (ASO) were evaluated by 2-dimensional, M-mode, Doppler, and pulsed Tissue Doppler. These patients were compared with 33 healthy children of the same age and gender as the normal control group. Student's t-test and Pearson correlation were used to analyze the data. Besides, P<0.05 was considered as statistically significant. RESULTS: The mean follow up time was 40.9±5.6 months. Among the 33 patients with ASO, 6% had mild pulmonary stenosis, while 3% had mild pulmonary insufficiency. Aortic stenosis and aortic insufficiency of trivial to mild degree was seen in 12% and 12% of the patients, respectively. The patients' systolic velocity of tricuspid (S), early diastolic velocity of tricuspid (Ea), and late velocity of tricuspid valve (Aa) were significantly different from those of the controls (P<0.001). Also, pulmonary annulus diameter was significantly dilated in the patients compared to the controls (1.67±0.41 vs. 1.29±0.28, P≤0.001). Besides, aortic annulus diameter (1.56±0.42 vs. 1.24±0.21, P=0.001) and also aortic sinus diameter (2.06±0.41 vs. 1.44±0.34, P=0.002) were significantly dilated, while sinutuboar junction diameter (1.65±0.5 vs. 1.28±0.29, P=0.094) was not dilated. Left ventricular function was in the normal range. CONCLUSIONS: This study showed good left ventricular function, but some abnormalities in lateral tricuspid tissue Doppler velocities. Neoaortic and pulmonary diameters were significantly dilated, while aortic and pulmonary insufficiencies were clinically insignificant in most of the patients. Long-term follow-up is necessary in these patients.

Brucellosis relapse causing prosthetic valve endocarditis and aortic root infective pseudoaneurysm.

We report a previously treated case of brucellosis and aortic root replacement, which became complicated by prosthetic valve endocarditis and a massive aortic root pseudoaneurysm. Preoperative blood and intraoperative pseudoaneurysm wall cultures were positive for Brucella, and the patient was managed successfully with a combination of surgical and medical treatment. Brucella endocarditis is further discussed.