Camptodactyly-arthropathy-coxa vara-pericarditis syndrome and an unusual association with mitral stenosis.

BACKGROUND: Campotodactyly-artrhropathy-coxa vara-pericarditis (CACP) syndrome is a very rare autosomal recessive genetic disorder. It is characterized by flexion contracture of the fifth finger (camptodactyly); noninflammatory arthropathy; decreased angle between the shaft and the head of the femur (coxa vara) and pericarditis. Its association with mitral stenosis has not yet been reported. Hereby we report this unique association with CACP syndrome. CASE: An eleven-year-old girl presented with non-productive cough, dyspnea, and orthopnea. She was diagnosed CACP syndrome at the age of seven and a biallelic frameshift mutation in the PRG4 gene was determined. The physical examination revealed pectus excavatum, camptodactyly, genu valgum, tachypnea and orthopnea. The functional capacity was NYHA III-IV. She had 2/6 soft pansystolic murmur at 4th left intercostal space and a rumbling diastolic murmur at apex. Echocardiography revealed an enlarged left atrium, severe stenotic mitral valve with a mean diastolic transmitral gradient of 22.5 mmHg, mild mitral regurgitation and mild apical pericardial effusion. The patient had mitral comissurotomy and partial pericardiectomy operation. Her post-operative transmitral gradient decreased to 6.9 mmHg and the pulmonary pressure was 30 mmHg. Her functional capacity increased to NYHA I-II. CONCLUSIONS: The main defect is the proteoglycan 4 protein which acts like a lubricant in articular and visceral surfaces. Therefore, the leading clinical feature is arthropathy. Cardiac involvement other than clinically mild pericarditis is not usually expected. Three types of proteoglycans (decorin, biglycan, and versican) are present in the mitral valve. This could be the reason of mitral valve involvement in rare cases as like ours. It is important that these patients undergo echocardiographic examination regularly.

First Harvest of Pediatric and Congenital Heart Surgery Multicenter Database in Turkey: Novel Application of Real-Time Online Reporting.

OBJECTIVES: A new congenital heart surgery database (CKCV) with real-time online reporting function was recently developed in Turkey. All standard international parameters were used, but Aristotle Comprehensive Complexity score was modified. In this study, the first analysis of the CKCV Database is reported. METHODS: The CKCV Database included 2307 procedures from 12 centers between January 2018 and March 2020. All parameters, including 10 real-time online reports, which represent the number of centers, number and mortality rates of all procedures, number of extracorporeal membrane oxygenation (ECMO) and results, details of postoperative complications, age-group statistics, analysis for priority status, mean intensive care and hospital stay durations of the procedures, results of Aristotle Basic, Modified Aristotle Comprehensive (MACC) and Society of Thoracic Surgeons-European Association (STAT) Score Categories, comparison of centers were analyzed. RESULTS: Most common 10 procedures were ventricular septal defect (VSD) repair (n = 273), tetralogy of Fallot (TOF) repair (n = 243), atrial septal defect (ASD) repair (n = 181), complete AVSD repair (n = 95), cavopulmonary anastomosis (n = 81), systemic to pulmonary shunt (n = 79), modified Fontan (n = 71), subaortic resection, (n = 66) PA banding (n = 66), and arterial switch operation (n = 66). Cardiopulmonary bypass was used in 84.6% of the procedures. Overall mortality rate was 6.0%. A total of 618 major and 570 minor complications were observed in 333 and 412 patients, respectively. According to six MACC categories, number of the patients and mortality rates were I (293; 0.3%); II (713; 1.4%); III (601; 3.3%); IV (607; 12%); V (84; 35.7%); and VI (9; 55.6%), respectively. Analysis of five STAT Categories showed 0.7, 3.8, 5.4, 14.9, and 54.7% mortality rates. CONCLUSIONS: CKCV Database has a great potential for nationwide quality improvement studies. Users could instantly analyze and compare their results to national and international aggregate data using a real-time online reporting function. This is the first multicenter congenital database study in Turkey.

Noninvasive monitoring of central venous oxygen saturation by jugular transcutaneous near-infrared spectroscopy in pediatric patients undergoing congenital cardiac surgery

Background and aim: In patients undergoing congenital cardiac surgery, it is crucial to maintain oxygen demand-consumption balance. Central venous oxygen saturation (ScvO2) is a useful indicator of oxygen demand and consumption balance which is an invasive method. Near-infrared spectroscopy (NIRS) is a noninvasive, continuous monitoring technique that measures regional tissue oxygenation. NIRS that is placed over the internal jugular vein cutaneous area (NIRSijv) has the potential to show ScvO2 indirectly. In this study, we aimed to determine the correlation between ScvO2 with NIRSijv in pediatric patients undergoing congenital cardiac surgery. Materials and methods: Fifty children participated in the study. Four patients were excluded for the inability of internal jugular vein (IJV) catheterization due to technical difficulties. After anesthesia induction, NIRS probes were placed on the IJV site with ultrasound guidance for the measurement of continuous transcutaneous oxygen saturation. The catheter insertion was also done through the IJV from the other side using ultrasound guidance. Cerebral oxygenation monitoring was done using NIRS with a single pediatric probe placed on the right forehead. Values of NIRSijv, cerebral NIRS (NIRSc) and ScvO2, were recorded at certain times until postoperative 24th hour. Results: Data were collected at 8 different time points. There was a significant correlation between ScvO2 and NIRSijv in all measurement time points (r = 0.91), (P = 0.001). The mean bias between ScvO2 and NIRSijv was 2.92% and the limits of agreement were from 11% to ­5.2%. There was a moderate correlation between ScvO2 and NIRSc (r = 0.45), (P = 0.001). The mean bias between ScvO2 and NIRSc was 2.7% and the limits of agreement were from +26% to ­20%. Conclusions: In this study, we found a strong correlation between ScvO2 and NIRS measurements taken from the internal jugular vein site. Accordingly, continuous noninvasive monitoring with transcutaneous NIRSijv can be an alternative method as a trend monitor for the central venous oxygen saturation in pediatric cardiac patients undergoing congenital cardiac surgery.

Comparison of bilateral cerebral and somatic tissue oxygenation with near-infrared spectroscopy in cyanotic and acyanotic pediatric patients receiving cardiac surgery.

INTRODUCTION: Compromise of tissue oxygenation during surgery is associated with increased mortality and morbidity in the postoperative period in patients with congenital cardiac disorders. It may be monitored with near-infrared spectroscopy (NIRS). We aimed to evaluate the tissue oxygenation and factors which may affect it by bilateral cerebral and somatic NIRS levels during cardiopulmonary bypass and to compare the NIRS values of cyanotic and acyanotic patient groups. MATERIAL AND METHODS: Two groups of patients with cyanotic and acyanotic congenital heart diseases were included in the study. Each group consisted of 15 patients between 0 and 5 years of age. All data were collected following anesthesia induction (T1), the 10th (T2) and 30th min (T3) of cardiopulmonary bypass (CPB), every 30 min during CPB (T4, T5, T6) and 1 h after (TS). Bilateral and somatic NIRS, blood gases, mean arterial pressure, and temperatures were recorded. RESULTS: Left and right somatic NIRS values in groups at all measurements did not differ significantly. Left and right cerebral NIRS values at T2 and T3 in cyanotic patients were significantly higher than in acyanotic patients. Mean arterial pressure and lactate levels at T1 and T3 measurements were responsible for left cerebral NIRS changes and mean arterial pressure on right cerebral NIRS values. CONCLUSIONS: Monitorization of tissue perfusion has critical importance during CPB of patients with congenital heart defects. Oxygenation may easily and reliably be measured with NIRS. Cerebral and somatic NIRS are more pronounced in cyanotic patients and cerebral NIRS is strongly associated with mean arterial pressure and circulating lactate levels.

Atrial septal defect closure via mini-thoracotomy in pediatric patients: Postoperative analgesic effect of intercostal nerve block.

BACKGROUND: In this study, we evaluated the efficacy of intercostal nerve block for postoperative pain management in pediatric patients undergoing atrial septal defect closure through a right lateral mini-thoracotomy. METHODS: Between January 2016 and January 2019, a total of 63 pediatric patients (37 males, 26 females; mean age 34.8±26.8 months; range, 2 to 96 months) who underwent corrective congenital heart surgery for atrial septal defect closure through a right lateral mini-thoracotomy were retrospectively reviewed. The patients were divided into two groups as those (Group 1, n=33) receiving intercostal nerve block and general anesthesia and those (Group 2, n=30) receiving general anesthesia alone. Intravenous morphine at a dose of 0.03 mg/kg was applied as rescue analgesia to the patients with a Ramsay Sedation Scale score of >4 and Children"s Hospital of Eastern Ontario Pain Scale score of >7. The total analgesic requirement, adverse effects, duration of mechanical ventilation and length of stay in the intensive care unit were recorded. RESULTS: The mean duration of mechanical ventilation and intensive care unit stay was shorter in Group 1 compared to Group 2 (3.6±1.3 vs. 9.4±2.1 h; 23±2.6 vs. 30±7.2 h, respectively) (p<0.0001). The need for postoperative rescue analgesia was statistically significantly lower in Group 1 compared to Group 2 (0.3±0.5 mg vs. 1.1±0.9 mg, respectively) (p=0.003). The mean total morphine consumption was also lower in Group 1 compared to Group 2 (4.0±2.2 mg vs. 9.0±3.4 mg, respectively) (p<0.0001). CONCLUSION: Intercostal nerve block before thoracotomy closure in pediatric patients undergoing atrial septal defect repair under mini-thoracotomy provides early extubation, shorter mechanical ventilation duration and intensive care unit stay, and reduced analgesic requirements.

Why national databases are necessary for pediatric and congenital heart surgery practice?

In international experience, performance improvement, patient safety, and quality assurance are mainly based on database studies in the field of pediatric and congenital cardiac surgery. Data collection and analysis of the databases allow the clinician to identify the complexity, to predict possible risks and complications, and to appropriately evaluate the outcomes and performances. Recently, the Children"s Heart Foundation Working Group has developed a database project in Turkey based on the parameters and methodologies of the international databases, namely the Pediatric Heart Surgery National Database. Currently, it is a must for us to put this project into practice to reach the global standards in the pediatric and congenital heart surgery in our country. We believe that all children with congenital heart disease should have an opportunity to benefit from the most recent medical and surgical treatment modalities with the most favorable results.

Surgical Repair of Congenital Left Atrial Aneurysm and Mitral Valve Insufficiency in a Four-Year-Old Child.

Left atrial aneurysm is an extremely rare anomaly, which can be associated with supraventricular arrhythmia, compression of coronary arteries, intracardiac thrombus, life-threatening systemic embolization, pulmonary venous obstruction, mitral valve insufficiency, and congestive heart failure. Herein, we report a four-year-old boy who had a giant aneurysm of the left atrium and severe mitral regurgitation. The aneurysm and mitral valve cleft causing severe mitral regurgitation were successfully repaired.

An Easy Modification of Tube Graft Interposition During Fenestration in Borderline Fontan Circulation.

One of the options for the management of borderline/failing extracardiac Fontan circulation is surgical creation of an atrial fenestration to decompress the systemic venous compartment and improve cardiac output. Depending on the body surface area of the patient, a 5- to 10-mm polytetrafluoroethylene (PTFE) tube graft can be used. When fenestration is required in a patient with failing Fontan circulation, particularly in redo cases, application of a side-biting clamp may be challenging because of adhesions and a thickened atrial wall. In this article, we present our off-pump technique of atrial-side anastomosis of PTFE graft interposition between an extracardiac Fontan conduit and the atrium without using a side-biting clamp.

Indications and risks of delayed sternal closure after open heart surgery in neonates and early infants.

BACKGROUND: Delayed sternal closure (DSC) has been an essential part of neonatal and infant heart surgery. Here, we report our single institution experience of DSC for eight years. METHODS: The successive 188 patients were analyzed retrospectively. Sternum was closed at the end of the operation in 97 (51.6%) patients (primary sternal closure [PSC] group). Sternum was left open in 91 (48.4%) patients. Among them, 45 (23.9%) had only skin closure (DSCs group) and 46 (24.4%) had membrane patch closure (DSC membrane [DSCm] group). Median age was higher in PSC group (90 days) than DSCs (11 days) and DSCm groups (9.5 days). RESULTS: Mortality was 1%, 11.1%, and 28.2% in PSC, DSCs, and DSCm groups, respectively (P < .05). Univariate analysis recognized the neonatal age (odds ratio [OR] = 4.2), preoperative critical condition (OR = 5.3), cardiopulmonary bypass time >180 minutes (OR = 4), and cross clamp time >99 minutes (OR = 3.9) as risk factors for mortality. Total morbidity rate was higher in DSCm group (73.9%) than DSCs group (51.1%) and PSC group (23.7%; P < .001). Mechanical ventilation time, intensive care unit stay, and hospital stay were longer in DSCs and DSCm groups than PSC group (P < .001). The incidence of hospital infection was also higher in DSCs (43.5%) and DSCm (33.3%) groups than PSC group (20.6%; P < .05). But there was no difference in the incidence of sternal wound complications, including both deep and superficial (4.1%, 8.8%, and 4.4%, respectively). CONCLUSION: Although the risk of sternal wound complications is not different, patients who necessitate DSC (using both skin and membrane closure techniques) have more complicated postoperative course than patients with PSC.

Challenges in the management of patients with functionally univentricular heart in Turkey.

Management of patients with functionally univentricular heart encompasses a wide array of developments over the years in every country. This article describes our working group experiences and 30-year story of single ventricle surgery in Turkey. Diagnosis, surgical treatment, and medical treatment of this complex group of patients necessitate courageous and continuous team effort with multi-institutional collaboration.

Surgical repair of a cause of sudden death: left coronary artery originating from right coronary sinus.

Left coronary artery originating from the right coronary sinus is one of the most frequent causes of sudden death in young people. We present a reconstructive surgical technique for left coronary artery and main pulmonary artery in a case with anomalous origin of the left coronary artery from the right coronary sinus. A 15-year-old boy underwent unroofing of the left main coronary artery and patch arterioplasty with autologous pericardium after transection of the main pulmonary artery. The pulmonary artery was reconstructed with autologous pericardium and a piece of dacron patch in order to prevent coronary artery compression. This surgical approach resulted in successful clinical outcome.

[Pulmonary artery banding operation and results of terminal biventricular and univentricular repair]. / Pulmoner arter bant ameliyati ve sonrasinda biventriküler ve üniventriküler tamir sonuçlari.

OBJECTIVE: In this study, we report the current indications, early-midterm results and the outcome of pulmonary artery banding (PAB) operation after definitive repair. METHODS: Between 2000 and 2007, 28 infants underwent PAB operation. Ages were between 21 days and 6 months (mean 3.0+/-1.7 months). All patients had pulmonary hypertension at systemic level. Fourteen patients were candidates for terminal biventricular repair [atrioventricular septal defect (AVSD) (n=6), double outlet right ventricle (DORV) +ventricular septal defect (VSD) (n=4), Swiss cheese VSD (n=2), transposition of the great arteries (TGA) +Swiss cheese VSD (n=1), ASD+VSD+aortic coarctation (n=1)], and the remaining 14 were candidates for terminal univentricular repair [double inlet left ventricle (n=5), double inlet right ventricle (n=2), AVSD+left ventricular (LV) hypoplasia (n=2), DORV+LV hypoplasia (n=2); tricuspid atresia (n=1), left AV valve atresia (n=1), TGA+Swiss cheese VSD+LV hypoplasia (n=1). RESULTS: Three patients (1 in biventricular group; 2 in univentricular group) died in the early postoperative period (10.7%). Three patients needed long duration of mechanical ventilatory support. Twenty- three of the surviving patients (92%) were followed -up between 1 month to 7 years. Currently 7 patients underwent successful biventricular repair and five patients underwent univentricular repair (extracardiac Fontan 3; Glenn 2). Two patients died early and 1 patient died 1 year after extracardiac Fontan operation. Survival for biventricular group was 92.8% at 1 and 4 years and 85.7% and 58.4% respectively for univentricular group (p<0.05). CONCLUSION: Pulmonary artery banding operation has still a significant role in the palliation of certain congenital cardiac anomalies. Outcome of patients who are candidates for biventricular repair is better than the univentricular repairs.

[Evaluation of surgical approaches and early and midterm results of treatment for atrioventricular septal defect]. / Atriyoventriküler septal defekt tedavisinde cerrahi yaklasimlarin ve erken ve orta dönem sonuçlarin degerlendirilmesi.

OBJECTIVES: We evaluated patients who underwent complete or partial surgical correction for atrioventricular septal defect (AVSD) with regard to surgical techniques and early and midterm results. STUDY DESIGN: Forty-six patients were treated for complete (n=28) or partial (n=18) AVSD between 2000 and 2007. There were nine boys and 19 girls (mean age 5.5 months; range 1.5 to 11 months) with complete AVSD. Of these, 17 patients underwent total repair, while 11 patients underwent palliative procedures. Five males and 13 females (mean age 11 years; range 1 to 50 years) with partial AVSD were treated with total repair. Down syndrome was seen in nine patients (32.1%) and one patient (5.6%) in complete and partial AVSD groups, respectively. Twenty-one patients (75%) and 14 patients (77.8%) could be followed-up for a mean of 26.3 months (range 1-72) and 21.8 months (range 2 to 71) in the two groups, respectively. RESULTS: Total repair of partial AVSD resulted in no mortality or significant morbidity. Early postoperative mortality occurred in three cases (10.7%) after repair of complete AVSD, one of which had Down syndrome. Six patients required prolonged mechanical ventilation beyond one week. Two patients without Down syndrome underwent reoperation due to severe atrioventricular (AV) valve insufficiency in the early postoperative period. None of the patients required permanent pacemaker implantation. Clinical and echocardiographic monitoring showed moderate left AV valve insufficiency in three patients in each group, while the remaining patients had no or minimal insufficiency. CONCLUSION: Total repair of complete AVSD should be the procedure of choice in early infancy. Left AV valve insufficiency continues to be the most important cause of postoperative morbidity in these cases.

[Combination of complete atrioventricular septal defect and tetralogy of Fallot: surgical management and its results]. / Komplet atriyoventriküler septal defekt ve Fallot tetralojisinin birlikte bulundugu hastalarda cerrahi yaklasim ve sonuçlar.

OBJECTIVES: Complete atrioventricular septal defect (AVSD) with tetralogy of Fallot (TOF) is a rare congenital heart anomaly. We evaluated surgical results of total repair in patients with TOF and AVSD. STUDY DESIGN: Seven patients (5 girls, 2 boys; age range 2.5 to 14 years) underwent total repair for AVSD and TOF. Three patients had Down syndrome. Three patients had previous systemic-to-pulmonary shunt operations. Accompanying anomalies were left superior vena cava (n=2), left atrial isomerism (n=1), muscular VSD (n=1), and double outlet right ventricle (n=1). Preoperative diagnoses were based on echocardiographic examinations. The two-patch technique was used for surgical repair. Reconstruction of the right ventricular outflow tract was performed using transannular and infundibular patches in four and three patients, respectively. The follow-up period ranged from six months to nine years (mean 3.4+/-2.9 years). RESULTS: No mortality occurred throughout the follow-up period. Two patients had prolonged hospitalization (>1 month) due to pulmonary infection and sepsis. At the latest follow-up, functional capacity was NYHA class I in five patients, and class II in two patients. Final echocardiographic examinations showed mild left atrioventricular (AV) valve insufficiency in five patients, and mild (n=3) or moderate (n=1) right AV valve insufficiency. Two patients had mild residual pulmonary stenosis, and three patients with a transannular patch had free pulmonary insufficiency. All the patients had proper right and left ventricular functions and all were in sinus rhythm. CONCLUSION: With a proper surgical strategy and technique, AVSD and TOF can be corrected successfully. Long-term follow-up is necessary for AV valve dysfunction and pulmonary insufficiency.

[Early-mid term results of the one and a half ventricle repair]. / Bir buçuk ventrikül tamirinin erken-orta dönem sonuçlari.

OBJECTIVE: The aim of this study is to investigate the early and mid term results of one and a half ventricle repair. METHODS: Pathological and demographic data, prior palliative operations selection criteria and operative properties of six patients with right ventricular hypoplasia undergoing one and a half ventricle repair were investigated. Patients were followed for mean 55.6+/-18.9 months by clinical and echocardiographic examinations. RESULTS: One patient died in the early-mid term follow-up period. The intensive care unit and hospitalization mean time were 2.8+/- 0.83 and 11.25+/- 6.55 days respectively. One patient had recurrent pleural effusion; she also had epicardial permanent pace implantation for atrioventricular block within the postoperative second month. Another patient was reoperated for important tricuspid and pulmonic valve regurgitation at postoperative 3rd year. All other patients were followed with medical therapy in NYHA class I-II status. CONCLUSION: The early and mid-term results of one and a half ventricle repair seem to be acceptable. This method can be a good alternative to Fontan repair in suitable patients.

Total autologous Ross procedure in a child with aortic root abscess.

BACKGROUND AND AIM OF THE STUDY: Endocarditis with aortic root abscess is one of the most complicated surgical problems. METHODS: An 8-year-old girl presented with dyspnea, high fever, and fatigue. She had stenotic bicuspid aortic valve with endocarditis and aortic root abscess. Ross procedure was performed with fresh autologous pericardial tube and pericardial monocusp valve. Right internal mammary artery to right coronary artery bypass was also done due to destroyed right coronary artery ostium. RESULTS: Four years after the operation she is in excellent clinical condition without medications. Echocardiography reveals mild autograft regurgitation and mildly stenotic right ventricular outflow tract. CONCLUSIONS: If homografts are not available, total reconstruction of RVOT with autologous fresh pericardium may offer reasonable early and mid-term results especially when active endocarditis and aortic root abscess is involved.

Stentless heterograft conduits for pulmonic ventricle to pulmonary artery connection: is it the right option?

BACKGROUND AND AIM OF THE STUDY: A variety of stentless heterograft conduits has been developed as alternatives for pulmonary outflow reconstruction. Herein are reported the authors' results with four different types of stentless heterograft conduit implanted during the past nine years. METHODS: Between January 1996 and March 2005, stentless heterograft conduits were used in 54 patients (mean age 9.9 +/- 7.5 years; median age 7 years; range: 1-32 years) to reconstruct the pulmonary outflow tract. The main pathologies were tetralogy of Fallot, transposition of the great arteries and aortic valve disease (Ross operation). A Baxter-Edwards Prima valve (EP) was used in three patients, a Medtronic Freestyle bioprosthesis (MF) in 27, a Cryolife Ross pulmonic heterograft (CR) in 14, and a Medtronic Contegra bovine jugular vein conduit (MC) in 10. The mean conduit diameter was 20.4 +/- 1.7 mm (range: 16-23 mm). RESULTS: Early mortality was 5.5%. Mean intensive care unit and hospital stays were 4.3 +/- 4.8 days and 11.7 +/- 7.2 days, respectively. Clinical follow up was complete for 92.1% of patients; the mean follow up period was 45.6 +/- 20 months. Excluding early deaths, the probability of patient survival (Kaplan-Meier) was 97.9% and 74.6% at one and five years, respectively. Transconduit gradients increased significantly in all conduit types during follow up. Conduit failure occurred in 15 patients (two EP, nine MF, three CR, one MC) at a median duration of 70 +/- 7 months (range: 56-84 months). To date, nine patients have undergone conduit re-replacement, without mortality and major morbidity. The probability of freedom from conduit failure was 91.6%, 66.4% and 27.9% at three, five and seven years, respectively. CONCLUSION: Stentless heterograft conduits are suitable alternatives to homografts, with acceptable early and mid-term results. However, they do not offer any significant advantage over homografts or other conventional Dacron or pericardial conduits.

A rare cause of respiratory distress in infants: tracheal compression due to anomalous course of innominate artery.

Incomplete vascular ring is a rare congenital vascular anomaly causing tracheal compression. A case of anomalous innominate artery causing tracheal compression and low grade tracheomalacia with remarkable dyspnea and intermittent apnea in a five-month-old infant is reported. Chest X-ray revealed enlarged upper mediastinum. Patent foramen ovale and thymus hyperplasia were detected on echocardiographic examination. Angiography demonstrated left aortic arch with normal arterial branching pattern. Thorax computerized tomography (CT) revealed tracheal compression of more than 50% at innominate artery level and aberrant position of the hyperplastic thymus. Operation was planned for subtotal thymectomy and relocation of innominate artery in order to relieve tracheal obstruction. Postoperative thorax CT demonstrated satisfactory relief of tracheal compression. Patient was discharged from hospital on sixth postoperative day in good condition. On clinical follow-up he is completely asymptomatic without any signs of dyspnea or intermittent reflex apnea. In severely symptomatic cases, surgical treatment offers excellent results.