Neonatal Outcomes of Critical Congenital Heart Defects: A Multicenter Epidemiological Study of Turkish Neonatal Society : Neonatal Outcomes of CCHD.

Critical congenital heart disease (CCHD) is one of the leading causes of neonatal and infant mortality. We aimed to elucidate the epidemiology, spectrum, and outcome of neonatal CCHD in Türkiye. This was a multicenter epidemiological study of neonates with CCHD conducted from October 2021 to November 2022 at national tertiary health centers. Data from 488 neonatal CCHD patients from nine centers were entered into the Trials-Network online registry system during the study period. Transposition of great arteria was the most common neonatal CHD, accounting for 19.5% of all cases. Sixty-three (12.9%) patients had extra-cardiac congenital anomalies. A total of 325 patients underwent cardiac surgery. Aortic arch repair (29.5%), arterial switch (25.5%), and modified Blalock-Taussig shunt (13.2%). Overall, in-hospital mortality was 20.1% with postoperative mortality of 19.6%. Multivariate analysis showed that the need of prostaglandin E1 before intervention, higher VIS (> 17.5), the presence of major postoperative complications, and the need for early postoperative extracorporeal membrane oxygenation were the main risk factors for mortality. The mortality rate of CCHD in our country remains high, although it varies by health center. Further research needs to be conducted to determine long-term outcomes for this vulnerable population.

Urgent double switch operation in a patient with congenitally corrected transposition of great arteries and an untrained systemic ventricle.

Congenitally corrected transposition of the great arteries is a rare cardiac abnormality. A nine-month-old male infant with the diagnosis of congenitally corrected transposition of the great arteries was admitted to our clinic with a history of pulmonary artery banding to train the left ventricle three months ago. On admission, he presented with bronchopneumonia, cyanosis, dyspnea, and severe biventricular heart failure associated with significant valvular regurgitation. An urgent double switch operation was performed with atrial and arterial switch procedures. Eight days of mechanical circulatory support was deemed mandatory following surgery. The patient is still under follow-up uneventfully three years after the operation.

A patient with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) and 13 live births.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) or Bland-White-Garland syndrome is a rarely seen congenital anomaly. Adult and infantile types are defined according to the degree of collateral development between the left coronary artery (LCA) and right coronary artery (RCA). If left untreated, ALCAPA has a 90% mortality rate in the first year of life, primarily due to myocardial ischaemia and heart failure. The degree of collateral development and the related LCA perfusion in ALCAPA syndrome determine the occurrence of symptoms. Herein, we present a case of a female patient who had previously, without any symptoms, given live birth to 13 babies. She had been experiencing exertional angina, which started long after the delivery of her 13th child. Since our patient had well-developed collaterals to the LCA, she was asymptomatic and able to give birth to the children via the vaginal route without any problems. Having well-formed collateral vessels between the RCA and LCA may prevent patients from developing symptoms, and even stressful conditions such as pregnancy may be tolerable.

A better approach for left ventricular training in transposition of the great arteries and intact interventricular septum: Bidirectional cavopulmonary anastomosis and pulmonary artery banding.

OBJECTIVE: Management of the patients with transposition of the great arteries and intact ventricular septum may be challenging beyond the newborn period. Herein, we would like to present our alternative strategy for training the left ventricle in these patients. METHODS: Six patients with transposition of the great arteries and intact ventricular septum were evaluated in our clinic. Two of them were palliated with Glenn procedure and pulmonary banding as a definitive treatment strategy at other centers. Four patients were operated on and a bidirectional cavopulmonary anastomosis in combination with pulmonary artery banding was performed (stage-1: palliation and ventricular training) in our center. In four out of these six patients, arterial switch operation was performed with takedown and direct re-anastomosis of the superior vena cava to right atrium after an interstage period of 21-30 months (stage-2: anatomical repair). RESULTS: Any mortality was not encountered. The left ventricular mass indices increased from 18-32 to 44-74 g/m2 in patients undergoing the anatomical repair. All of the patients were uneventfully discharged following the second stage. The mean follow-up period was 20 months (9-32 months) following stage 2. All of the patients are doing well with trivial neoaortic regurgitation and normal biventricular function. CONCLUSIONS: Bidirectional cavopulmonary anastomosis with pulmonary artery banding may be a promising left ventricle training approach in ventriculoarterial discordance when compared to the traditional pulmonary artery banding with concomitant systemic-to-pulmonary artery shunt procedures which still carry a significant interstage morbidity and mortality.

Ninety-two days of extracorporeal membrane oxygenation support following congenital heart surgery.

We report on a case involving a 10-month-old infant who received prolonged ECMO therapy following cardiac surgery for multiple ventricular septal defects (VSD). The patient was successfully weaned from 92 days of ECMO support without any long-term deficits.

Bedside PDA ligation in premature infants less than 28 weeks and 1000 grams.

BACKGROUND: PDA(Patent ductus arteriosus) is a common and clinically important condition which is presented with a number of hemodynamic and respiratory problems such as intraventricular hemorrhage, pulmonary hemorrhage and necrotizing enterocolitis due to increased pulmonary blood flow and stealing from systemic circulation. The incidence of PDA among the infants that were born before the 28th gestational week is as high as 70 %; and spontaneous closure rates in very-low-birth-weight premature neonates(VLBWPN) is around 34 %. The onset, duration, and repeat number of consecutive courses of the prostaglandin synthesis inhibitor medication for PDA closure are still issues of debate. Bed-side PDA closure is a safe surgical procedure in both mature and premature babies. Here we aim to retrospectively present our 26 cases which were less than 28 weeks and 1000 grams that underwent bed-side PDA ligation. METHODS: This retrospective study included 26 VLBWPN with PDA that underwent bed-side ligation between 2012 and 2015. Babies were born before the 28th gestational week (23-27 weeks) and less than 1000 grams (489-970 gr). Of the 26, 15 were female and 11 were male. Indomethacin was administered to all of the cases as the medical closing agent. The medication was stopped due to unwanted effects in 6 cases. All of the patients took medical treatment before surgery. RESULTS: No surgical mortality occurred during our study. One case of pneumothorax was recorded as late surgical complication. Five of the 26 patients were lost, and the most common cause of mortality was sepsis (in 3 cases). The remaining 21 cases were discharged on days 86-238. The follow-up periods of the patients were 2 moths - 3 years. The most frequent problems encountered after discharge was chronic lung problems. CONCLUSIONS: Bed side PDA ligation surgery in the ICU is a safe method for VLBWPN with clinically significant PDA.

Double Switch Procedure and Surgical Alternatives for the Treatment of Congenitally Corrected Transposition of the Great Arteries.

BACKGROUND: We present our experience with the double switch operation in sixteen patients with congenitally corrected transposition of the great arteries. METHODS: We enrolled 16 patients with congenitally corrected transposition of the great arteries operated by a single surgeon between 1995 and 2015. The mean age was 25 months (range 4 to 72 months) and the mean body weight was 8.9 kg (range 4.3 to 19 kg) at the time of operation. RESULTS: We encountered seven patients with moderate to severe tricuspid regurgitation, five of which had Ebstein anomaly. We performed a combination of atrial and arterial switch procedures in 11 cases, one of which had a concomitant coarctation of the aorta that was repaired along with the double switch procedure. Atrial switch and the Rastelli procedures were performed in three cases with concomitant pulmonary stenosis. A combination of arterial switch, Hemi-Mustard procedure, and bidirectional cavopulmonary anastomosis was performed in two cases. During a mean follow-up period of 67 months (range three months to 18 years), we encountered one early postoperative mortality related to intracerebral bleeding. All but one of the patients are now in NYHA class I-II. CONCLUSIONS: Congenitally corrected transposition of the great arteries is a rare congenital cardiac anomaly in which the results of the anatomical repair with double switch operation appear to be superior to that achieved by a physiological repair.

Modified Single Versus Double-Patch Technique for the Repair of Complete Atrioventricular Septal Defect.

OBJECTIVES: We present our single center results of two commonly used surgical techniques, the modified single-patch and double-patch technique for the repair of the complete atrioventricular septal defect. METHODS: We enrolled 49 patients with complete atrioventricular septal defect who were operated by a single surgeon between 2004 and 2014. The modified single-patch technique was performed in 32 cases (group S), whereas the defect was repaired with the double-patch technique in 17 patients (group D). RESULTS: The mean age at the time of operation was 7.7 ± 8.6 (range 1-48) months and 9.9 ± 12.5 (range 1.5-48) months, in groups S and D, respectively. Mean body weight was 6.7 ± 3 (range 3.5-15.5) kilograms and 7.2 ± 3.8 (range 4.3-14.5) kilograms in groups S and D, respectively. Cross-clamp and cardiopulmonary bypass times were shorter in the modified single-patch group (65.6 ± 16.3 vs. 98.7 ± 19.8 minutes, p = 0.0001; 88.9 ± 23.3 vs. 128.9 ± 28.0 minutes, p = 0.0001). Postoperative atrioventricular valve function was improved in both groups. Mean follow-up period was 4.4 ± 3.2 (range 1.2-9.9) years. One patient in each group underwent reoperation for left atrioventricular valve insufficiency. We encountered two early and one late mortalities in modified single-patch group. One early mortality was experienced in the unmodified group. CONCLUSIONS: The modified single-patch and two-patch techniques have comparable results; however, the modified single-patch technique is performed with significantly shorter cross-clamp and cardiopulmonary bypass times, therefore we have adopted this technique on a routine basis for the treatment of the complete atrioventricular septal defect in our institute.

Mid-Term Results of Congenital Supravalvar Mitral Ring Resection.

BACKGROUND: Supravalvar mitral ring is a rare congenital anomaly leading to left ventricle inflow obstruction. We present our surgical experience and mid-term results in patients operated for supravalvar mitral ring and associated congenital heart defects. MATERIALS AND METHODS: A retrospective analysis was performed in order to evaluate the cases surgically treated with the diagnosis of supravalvar mitral ring between 2001 and 2014. Ten patients were identified, seven of whom had accompanying congenital heart defects. Median age at the operation was 4.5 years. RESULTS: Ventricular septal defects were encountered in half of the cases. Two of the patients had mitral annular hypoplasia, another two had the components of Shone's complex. The mean preoperative gradient across the supravalvar mitral ring decreased from 14.1 ± 4.2 mmHg to 4.6 ± 2.2 mmHg. All patients are alive and remain in a good clinical condition after a mean follow-up of five years. CONCLUSION: Supravalvar mitral ring is a surgically treatable cause of left ventricular inflow obstruction. Although residual gradients may be encountered in patients with mitral annular hypoplasia, surgical resection of the ring is encouraged.

Repair of unilateral absence of right pulmonary artery with contralateral pulmonary artery autograft interposition in an infant.

Unilateral absence of the pulmonary artery (PA) is a rare congenital abnormality. Although a traditional 2-stage approach with a preceding systemic-to-PA shunt followed by a definitive repair is mandatory in patients with absent extrapericardial pulmonary arteries, more creative solutions are necessary when 1-stage repair is concerned. We present a novel technique for repairing the absent intrapericardial right pulmonary artery (RPA) with a contralateral PA autograft segment interposition. We believe that this technique using solely autologous tissue is effective in patients in whom the intrapericardial segment of the PAs is absent.

Absence of left pulmonary artery in tetralogy of Fallot with absent pulmonary valve syndrome.

The rare combination of tetralogy of Fallot (TOF), absent pulmonary valve syndrome (APVS), and absent left pulmonary artery (ALPA) is reviewed herein. Children with TOF with APVS and ALPA should be closely monitored, even if they are asymptomatic or mildly symptomatic, and should undergo elective surgery at the end of infancy.

Surgical correction of cor triatriatum sinister in the paediatric population: mid-term results in 15 cases.

OBJECTIVES: Cor triatriatum sinister (CTS) is a rare developmental cardiac abnormality resulting in left ventricular inflow obstruction. In this report, we aimed to present our mid-term results of early childhood patients operated for CTS and associated cardiac abnormalities. METHODS: We enrolled 15 patients with CTS who were operated by a single surgeon between 2001 and 2013. A retrospective analysis was performed in order to determine the demographics, operative and postoperative results of the patients. The median age was 14 months and the median body weight was 8.2 kg at the time of operation. RESULTS: Fourteen patients had concomitant cardiac pathology. Three of the patients had atrial septal defect and 1 of the patients had partial abnormal pulmonary venous connection, whereas 4 of the patients had both. In 2 cases of complete atrioventricular septal defect, 1 case with ventricular septal defect, 1 case with patent ductus arteriosus, 1 case with double outlet right ventricle and another case with tetralogy of Fallot, complete repair was performed together with membrane resection in the left atrium (LA). The mean preoperative left atrial gradient was 13.3 mmHg, whereas the mean LA pressure at the first postoperative year was 4.2 mmHg. There was 1 case with early mortality due to septic multiorgan failure secondary to pneumonia. CONCLUSIONS: CTS is a rare congenital cardiac anomaly in which the results of the corrective surgery are encouraging. Early and long-term outcomes may be variable according to the associated congenital heart defects.

Aorta-to-Left Atrial Fistula Caused by Air Gun Pellet Cardiac Injury.

Abnormal communication between the ascending aorta and the cardiac chambers is rare, diverse in origin, and can be congenital or acquired. We report a case of a 10-year-old boy with acquired aorta-to-left atrial fistula associated with an air gun pellet injury and his successful treatment.

Atrial septal defect closure: comparison of vertical axillary minithoracotomy and median sternotomy.

BACKGROUND: This study aims to evaluate whether or not the method of right vertical axillary minithoracotomy (RVAM) is preferable to and as reliable as conventional sternotomy surgery, and also assesses its cosmetic results. METHODS: Thirty-three patients (7 males, 26 females) with atrial septal defect were admitted to the Cardiovascular Surgery Clinic of Cukurova University from December 2005 until January 2010. The patients' ages ranged from 3 to 22. Patients who underwent vertical axillary minithracotomy were assigned to group I, and those undergoing conventional sternotomy, to group II. Group I and group II were compared with regard to the preoperative, perioperative and postoperative variables. Group I included 12 females and 4 males with an average age of 16.5±9.7. Group II comprised 14 female and 3 male patients with an average age of 18.5±9.8 showing similar features and pathologies. The cases were in Class I-II according to the New York Heart Association (NYHA) Classification, and patients with other cardiac and systemic problems were not included in the study. The ratio of the systemic blood flow to the pulmonary blood flow (Qp/Qs) was 1.8±0.2. The average pulmonary artery pressure was 35±10 mmHg. Following the diagnosis, performing elective surgery was planned. RESULTS: No significant difference was detected in the average time of the patients' extraportal circulation, cross-clamp and surgery (p>0.05). In the early postoperative period of the cases, the duration of mechanical ventilator support, the drainage volume in the first 24 hours, and the hospitalization time in the intensive care unit were similar (p>0.05). Postoperative pains were evaluated together with narcotic analgesics taken intravenously or orally. While 7 cases (43.7%) in group I needed postoperative analgesics, 12 cases (70.6%) in group II needed them. No mortality or major morbidity has occurred in the patients. The incision style and sizes in all of the patients undergoing RVAM were preserved as they were at the beginning. Furthermore, the patients of group I were mobilized more quickly than the patients of group II. The patients of group I were quite pleased with the psychological and cosmetic results. No residual defects have been found in the early postoperative period and after the end of the follow-up periods. All of the patients achieved functional capacity per NYHA. No deformation of breast growth has been detected during 18 months of follow-up for the group I patients, who underwent RVAM. CONCLUSION: To conclude, the repair of atrial septal defect by RVAM, apart from the limited working zone for the surgeon in these pathologies as compared to sternotomymay be considered in terms of the outcomes, and early and late complications. And this has accounted for less need of analgesics and better cosmetic results in recent years.

Increased mean platelet volume is associated with coronary artery ectasia.

AIM: The present study was designed to investigate mean platelet volume (MPV) values in coronary artery ectasia (CAE) patients in comparison with individuals with coronary artery disease and normal coronary angiograms. MATERIAL AND METHODS: Patients with stable angina pectoris and scheduled for angiography were included in the study. Those with isolated coronary ectasia were evaluated in the coronary angiography. Mean platelet volume was measured in 126 patients (mean age: 57.2 ±11.3 years) with isolated CAE, 126 patients (mean age: 56.3 ±10.4 years) with coronary artery disease (CAD) and 122 control subjects (mean age: 58.1 ±11.1 years). Coronary artery ectasia was defined as lack of stenotic lesion, on visual assessment, of the coronary arteries with a luminal dilatation 1.5-fold or more of the adjacent normal coronary segments. Mean platelet volume values were recorded in all patients. Ectasia severity was evaluated and categorized in accordance with the Markis categorization. RESULTS: A significant difference with respect to age, sex, hypertension, diabetes mellitus, and cigarette use between the study groups was not found (p > 0.05). Mean platelet volume was significantly higher in patients with CAE and CAD than in the control group (9.8 ±3.8 fl and 9.7 ±3.9 fl vs. 8.8 ±3.3 fl, p < 0.001). There were no statistically significant differences between CAE and CAD groups. When type I and type IV subgroups were compared in patients with coronary artery ectasia, MPV was detected as statistically high in the type I subgroup (p = 0.026). CONCLUSIONS: It was shown that patients with CAE and CAD have higher MPVs than subjects with normal coronary angiograms. Moreover, it was detected that in coronary artery ectasias, the increase in MPV is related to both the atherosclerotic process and severity of the disease.