Plication for diaphragm paralysis after paediatric cardiac surgery: a single-centre experience.

OBJECTIVE: Diaphragm paralysis is a well-known complication following surgery for CHDs, which increases morbidity, mortality, and length of hospital stay as well as costs. Herein, we present our experience with diaphragm plication following paralysis of the phrenic nerve encountered after paediatric cardiac surgery. METHODS: This study retrospectively reviewed the medical records of 23 diaphragm plications in 20 patients who underwent paediatric cardiac surgery between January 2012 and January 2022. The patients were carefully selected based on aetiology and a combination of clinical manifestation and chest imaging characteristics including chest X-ray, ultrasonography, and fluoroscopy. RESULTS: Twenty-three successful plications were performed in 20 patients (15 males and 5 females) out of a total of 1938 operations performed in our centre. Mean age and body weight were 18.2 ± 17.1 months and 8.3 ± 3.7 kg, respectively. The period between the cardiac surgery and diaphragmatic plication was 18.7 ± 15.1 days. The highest incidence of diaphragm paralysis was encountered in systemic to pulmonary artery shunt patients with 7 out of 152 patients (4.6%). Any mortality was not encountered during a mean follow-up period of 4.3 ± 2.6 years. CONCLUSIONS: Early results of plication of the diaphragm following phrenic nerve palsy in symptomatic patients who underwent paediatric cardiac surgery are encouraging. Evaluation of the diaphragmatic function should be a routine part of post-operative echocardiography. Diaphragm paralysis may be a consequence of dissection, contusion, stretching, and thermal injury both in terms of hypothermia and hyperthermia.

Fontan procedure in patients with preoperative mean pulmonary artery pressure over 15 mmHg.

BACKGROUND: Several factors affect the long-term outcome of Fontan procedure, but a high pulmonary artery pressure is still one of the most important limitation for proceeding to a Fontan circulation. Herein, we present our experience in Fontan patients with high preoperative pulmonary artery pressures. METHODS: A retrospective analysis was performed to evaluate Fontan patients with a preoperative pulmonary artery pressure >15 mmHg between 2009 and 2020. Sixteen patients were operated on with a mean preoperative pulmonary artery pressure of 17.5 ± 2.1 mmHg. RESULTS: Mean age at the time of Fontan procedure was 7.8 ± 5.6 years. All the patients had stage 2 cavopulmonary anastomosis before Fontan completion, with a mean interstage period of 4 ± 2.6 years. Fontan completion was achieved with a polytetrafluorethylene tubular conduit, two of which were intra-extracardiac. Fenestration was performed in five (31%) cases. Postoperative pulmonary artery pressures and arterial oxygen saturation levels were 11.2 ± 2.8 and 97.8 ± 2 mmHg, respectively. Mean duration of pleural drainage was 3.9 ± 5.3 days. Any morbidity and mortality were not encountered during a mean follow-up period of 4.8 ± 7.7 years. CONCLUSIONS: The midterm results of stage 3 Fontan completion in patients with pulmonary artery >15 mmHg are encouraging. Not only the mean pulmonary artery pressure but also the pulmonary vascular resistance may be helpful in order to identify the high risk patients before Fontan completion.

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.

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.

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.

Severe mitral regurgitation due to anterior mitral leaflet perforation after surgical treatment of discrete subaortic stenosis.

Congenital subvalvular aortic stenosis may be associated with anomalies of the mitral valve. In this case, we present a patient with severe mitral valve regurgitation due to a perforation in the anterior mitral leaflet detected 4 months after an operation for relief of subaortic stenosis. A 10-year-old male patient who was operated for subvalvular aortic stenosis in another clinic was admitted to our hospital, and transthoracic echocardiography revealed severe mitral valve regurgitation due to a defect that was demonstrated at the anterior valve leaflet. The perforated area at the mitral valve zone A1 was repaired with a PTFE patch. The patient was successfully operated for the mitral valve perforation and the postoperative course was uneventful. In our case, the perforation in the anterior mitral leaflet implies a possible implementation of inappropriate surgical technique which necessitated a second surgical intervention after the initial operation.

Reverse anastomosis technique for the treatment of a rare coronary artery anomaly in D-transposition of the great arteries.

Arterial switch operation remains the gold standard surgical therapy for D-transposition of the great vessels. Coronary anomalies may complicate the surgical strategy and lead to an increased morbidity and mortality. However, as of today, even challenging coronary anomalies should not be a contraindication for the performance of complete repair. Here, we describe a technique for the translocation of an intramural, high-origin conal branch in a 5-month-old patient with D-transposition of the great vessels and ventricular septal defect.

Ross-Konno procedure for the surgical treatment of prosthesis-patient mismatch after prosthetic aortic valve replacement.

The Ross-Konno procedure has become a widely recognized surgical method for the treatment of complex left ventricular outflow tract obstruction (LVOTO) in patients of younger age. We applied this method in two young females who presented following mechanical aortic valve replacement with severe prosthesis-patient mismatch (PPM) resulting in significant gradient across the left ventricular outflow tract and congestive heart failure. In both cases the postoperative course was uneventful with immediate improvement in clinical symptoms. Both patients remain in excellent clinical and hemodynamic condition four and six years after the Ross-Konno procedure.

Extracorporeal membrane oxygenation for the treatment of acute respiratory failure due to respiratory syncytial virus after congenital heart surgery.

Mechanical circulatory support using extracorporeal membrane oxygenation is a therapeutic option for intractable cardiac or respiratory failure. It has widely been used for adult and pediatric patient population with considerable success in cases with otherwise dismal prognosis. We describe the successful use of extracorporeal membrane oxygenation in a 9-month-old patient suffering from a virus-related respiratory failure after congenital cardiac surgery.

Results for surgical closure of isolated ventricular septal defects in patients under one year of age.

BACKGROUND: This study evaluated the outcomes of patients undergoing surgical repair of isolated ventricular septal defect (VSD) in the first year of life with particular attention to age and severity of pulmonary hypertension (PH). METHODS: Between July 1, 2002 and May 31, 2012, 282 patients aged less than one year underwent isolated VSD closure at a median age of five months (range, 21 days to 1 year) and a median weight of 5.3 kg (range, 2.9 to 12.5 kg). Patients were divided into three groups according to the age at surgery (0-3, 3-6, and 6-12 months), and groups were compared in regard to severity of PH associated with morbidity and mortality. RESULTS: Four (1.4%) early and four (1.4%) late deaths occurred postoperatively. All mortalities were patients with severe PH, aged between 3 and 12 months. Although hemodynamic studies revealed a higher incidence of persistent postoperative PH in patients above three months of age, there was no statistically significant difference in morbidity associated with prolonged mechanical ventilation, and long intensive care unit and hospital stays between age-related groups. CONCLUSION: In this study, the incidence of mortality was higher in patients over three months of age undergoing repair of isolated VSD; the data suggest that the mortality may be decreased in patients with severe PH who were operated on earlier in life. We conclude that in infants with severe PH, early surgical repair (less than three months) of isolated VSDs is strongly advised to achieve more favorable results.

Results for surgical correction of complete atrioventricular septal defect: associations with age, surgical era, and technique.

BACKGROUND: This study was conducted to evaluate the outcomes of patients undergoing complete atrioventricular septal defect (CAVSD) repair with particular attention to age at surgery, surgical era, and technique. METHODS: One hundred and forty-seven patients undergoing CAVSD repair between November 2002 and February 2012 were grouped according to surgical era and technique. Group I (age: 9.4 ± 5.0 months; weight: 6.8 ± 1.7 kg) consisted of 45 patients, operated before August 2006, and was divided into subgroup Ia (31 patients; two-patch repair) and subgroup Ib (14 patients; modified single-patch repair). One hundred and two patients operated after August 2006 were included in Group II (age: 5.2 ± 3.1 months; weight: 4.9 ± 2.6 kg), and was divided into subgroup IIa (59 patients; two-patch repair) and subgroup IIb (43 patients; modified single-patch repair). Groups were compared with regard to perioperative variables and postoperative data. RESULTS: There were 19 early and five late deaths. Overall mortality was significantly higher in Group I, compared to Group II (p < 0.01). Comparison of Groups Ia to Ib and IIa to IIb revealed no statistically significant difference in mortality or morbidity. Age >8 months and preoperative common atrioventricular valve (CAVV) regurgitation ≥ moderate were significant risk factors for mortality and morbidity. After 40.8 ± 24.4 months, 99 (80.4%) of 123 (83.7%) survivors were asymptomatic without any medication, and 24 (19.5%) have mild symptoms. CONCLUSION: Our current results indicate that younger patient age and better preoperative CAVV functions were the main factors for a favorable outcome after surgical correction of CAVSD; and outcomes did not differ by the surgical technique.

Early and intermediate term results for surgical correction of total anomalous pulmonary venous connection.

BACKGROUND: This retrospective study evaluated factors associated with mortality and morbidity in patients having functionally biventricular heart (FBH) and functionally univentricular heart (FUH) undergoing total anomalous pulmonary venous connection (TAPVC) repair. METHODS: We reviewed medical records retrospectively and identified 72 patients undergoing TAPVC repair between July 2002 and December 2010; and 62 patients had FBH and 10 patients had FUH. The median age and weight at repair was 9.1 months (range, 8 days-16 years) and 6.7 kg (range, 2.5-57 kg). The TAPVC anatomy was supracardiac in 41 (56.9%), cardiac in 18 (25%), infracardiac in eight (11.1%), and mixed in five patients (6.9%). RESULTS: There were seven early deaths (2 FBH [25%] and 5 FUH [62.5%]) and one late death (FUH). Presence of FUH (p < 0.01), heterotaxy syndrome (p = 0.03), young age (p < 0.01), low weight (p < 0.01) and long CPB time (p = 0.04) at the time of surgery were statistically significant risk factors for mortality. Morbidity was related to long duration of postoperative mechanical ventilation, intensive care unit and hospital stay. Presence of FUH was the only significant risk factor for morbidity (p < 0.01). After a mean follow-up of 52.1 months (range, 3-74 months), 59 (92%) of 64 survivors were asymptomatic without any medication, and five have mild symptoms with regular physical activity. CONCLUSION: Operative results of TAPVC have dramatically improved in recent years. However, patients having FUH (especially with concomitant palliative surgery), heterotaxy syndrome, low weight, early age, and long CPB time still have an increased operative mortality.

Successful transcatheter closure of a Fontan fenestration with a bioabsorbable Biostar occluder.

We report the successful closure of an extracardiac Fontan fenestration with a bio-absorbable device, which may be refenestrated by a transcatheter route when needed, in a 10-year-old boy. The patient presented with cyanosis two years after an extracardiac Fontan operation. Echocardiography revealed a moderate shunt from the Fontan circulation into the systemic circulation with a mean pressure gradient of 3-4 mmHg. Treadmill testing revealed a significant decrease in oxygen saturation (down to the low 50's from a baseline level of 80-85%). Cardiac catheterization revealed normal pressure in the Fontan circuit. A temporary balloon occlusion test showed that the defect was suitable for permanent occlusion. The fenestration was then occluded by a bio-absorbable Biostar (NMT medical, Boston, USA) atrial septal occluder device. The oxygen saturation on room air increased up to 95% after closure.

Intermediate-term results after surgery for anomalous origin of the left coronary artery from the pulmonary artery.

PURPOSE: Anomalous origin of the left coronary artery from the pulmonary artery is optimally treated by creating a dual coronary system. Our aim was to review the results of operations performed in these patients and determine the intermediate-term outcomes for left ventricular function and mitral regurgitation. METHODS: Between July 2004 and January 2009 seven patients (5 boys, 2 girls) aged between 4 months and 12 years (median, 4.5 years) were operated for anomalous origin of the left coronary artery from the pulmonary artery. The surgical correction was either performed by direct implantation (58%) or restoration of a composite tunnel (42%). Simultaneous mitral annuloplasty was performed in one patient with severe mitral regurgitation and simultaneous total correction of tetralogy of Fallot was performed in another. RESULTS: There was no hospital or late deaths. Postoperative echocardiograms demonstrated a significant improvement in the left ventricular ejection fraction (52% ± 6% versus 39% ± 8%, P = 0.02) and mitral regurgitation (11% mild versus 48% moderate, P = 0.02) compared to those obtained preoperatively. CONCLUSION: Direct re implantation of the left coronary artery to the aorta and restoration of a composite tunnel from aortic and pulmonary artery walls are equally effective techniques with an acceptable operative mortality, excellent cardiac recovery, and intermediate survival.