Pulmonary artery banding and venous bidirectional cava-pulmonary shunt for two-stage arterial switch procedure in late referral of patients with transposition of the great arteries and intact ventricular septum: midterm results.

OBJECTIVE: Two-stage arterial switch operation and left ventricle retraining are necessary for the patients with left ventricle dysfunction and transposition of great vessels with intact ventricular septum (TGA-IVS) who are referred late. MATERIAL AND METHODS: Forty-seven patients with the diagnosis of TGA-IVS and left ventricle dysfunction who underwent arterial switch operation in our centre between July 2013 and August 2017 were analysed retrospectively. The inclusion criteria for left ventricle retraining were patients older than 2 months of age at presentation, having an echocardiographic left ventricle mass index of less than 35 g/m², and having an echocardiographic "banana-shaped" left ventricle geometric appearance. The patients were divided into two groups: pulmonary artery banding and Blalock Taussig shunt were performed as the initial surgical procedure for later arterial switch operation in Group I (n = 19) and pulmonary artery banding and bidirectional cava-pulmonary shunt in Group 2 (n = 28). RESULTS: The average age was found to be 122.3 ± 45.6 days in Group I and 145.9 ± 37.2 days in Group II. There was no statistically significant difference (p = 0.232 versus p = 0.373) between the average left ventricle mass index of the two groups neither before the first stage nor the second stage (26.6 ± 4.8 g/m² versus 25.0 ± 4.9 g/m² and 70.5 ± 12 g/m² versus 673.8 ± 12.0 g/m², respectively). The average time interval for the left ventricle to retrain was 97.7 ± 42.9 days for Group I and 117.3 ± 40.3 days for Group II, significantly lower in Group I (p = 0.027). The time spent in ICU, length of the period during which inotropic support was required, and the duration of hospital stay were significantly higher in Group I (p<0.001, p < 0.001, and p < 0.00, respectively). CONCLUSION: Pulmonary artery banding and bidirectional cava-pulmonary shunt can be performed as a safe and effective alternative to pulmonary artery banding and arterial Blalock Taussig shunt for patients with TGA-IVS in whom arterial switch operation is needed beyond the neonatal period. This approach involves a shorter hospital stay and fewer post-operative complications.

The use of basic parameters for monitoring the haemodynamic effects of midazolam and ketamine as opposed to propofol during cardiac catheterization.

OBJECTIVE: Our aim is to compare the haemodynamic and adverse effects of propofol versus the mixture of midazolam and ketamine as used in sedation for cardiac catheterization in children. METHODS: In a prospective randomized trial, we divided patients needing sedation into 72 receiving a mixture of midazolam and ketamine and 42 receiving propofol. Their ages ranged from 6 months to 12 years, and 1 year to 16 years, respectively. We collected data relative to heart rate, mean arterial pressure, respiratory rate, peripheral saturations of oxygen, and adverse effects. We assessed cyanotic patients to establish any relationship between the haemodynamic data and peripheral arterial saturations of oxygen. RESULTS: Demographic data, including age, gender, and weight, was not statistically different between the groups. In those receiving midazolam and ketamine, mean systemic arterial pressures before, and 30 minutes after, sedation were 64.3, with standard deviation of 9.8, and 62.5, with standard deviation of 10.2, millimetre of mercury (p equals to 0.237). Heart rates were 131.3, with standard deviation of 13.5, and 109.2, with standard deviation of 17.3 beats per minute, (p less than 0.001) whereas in those given propofol the comparable values were 71.2, with standard deviation of 14.4, and 53.6 with standard deviation of 9.7 millimetres of mercury (p less than 0.001), and 115.2, with standard deviation of 13.6, and 100.5 with standard deviation of 20.1 beats per minute (p less than 0.01), respectively. Mean systemic arterial pressures in the subgroups of cyanotic patients before and 30 minutes after sedation were 74.8, with standard deviation of 14.6, and 72.7, with standard deviation of 12.4 millimetres of mercury for those receiving midazolam and ketamine (p equals to 0.544), and heart rates were 119.3, with standard deviation of 12.2, and 104.6 with standard deviation of 16.1 beats per minute (p equals to 0.001). In those given propofol, the comparable values were 71.1 with deviation of 15.5 and 53.9 with deviation of 9.2 millimetres of mercury (p equals to 0.001), and 126.7 with deviation of 20.8 and 107.2 with deviation of 13.5 beats per minute (p equals to 0.001), respectively. CONCLUSIONS: In cyanotic children, propofol used as a sedative agent during cardiac catheterization causes a decrease in mean arterial pressure and arterial desaturation. Ketamine produces more stable haemodynamic data in children with congenitally malformed hearts.

Tissue Doppler imaging in rheumatic carditis.

OBJECTIVE: Our study was undertaken to assess cardiac functions by tissue Doppler echocardiography in patient with primary episode of rheumatic carditis. METHODS: We divided 82 patients with rheumatic carditis were divided in two groups; 50 patients with mild and 32 patients with mitral regurgitation of grade two or more. A control group consisted of 30 healthy children free of any disease. All children underwent conventional and tissue Doppler echocardiography initially and at the time of the follow-up examination. RESULTS: Myocardial systolic wave velocity of the mitral annulus was significantly higher in patients with mitral regurgitation of grade two or more when compared to the control group, but was not different between patients with mild mitral regurgitation and healthy subjects at the time of the initial attack. Myocardial precontraction time, myocardial contraction time, and the ratio of myocardial precontraction and contraction times were significantly prolonged, and the systolic myocardial velocity of the mitral annulus was significantly decreased in patients with mitral regurgitation of grade two or more at the time of the follow-up examination. The myocardial systolic wave velocity was significantly lower, and myocardial precontraction time, myocardial contraction time, and the ratio of the precontraction and contraction times, were significantly longer or greater between patients with grade two or more mitral regurgitation and the control group at follow-up examination. CONCLUSION: We detected subclinical systolic dysfunction of the left ventricle in children with a primary episode of rheumatic carditis due to ongoing ventricular volume overload. Tissue Doppler imaging provides a quantifiable indicator useful for cardiac monitoring of disease during the period of follow up.

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.

Crossed pulmonary arteries in conjunction with tetralogy of Fallot.

Crossed pulmonary arteries are an uncommon anomaly in which the left pulmonary artery originates superiorly and to the right of the right pulmonary artery, and the two pulmonary arteries cross each other. This anomaly may accompany other cardiac anomalies. We encountered this anomaly during echocardiographic examination of a 21-month-old boy. He had mesocardia, tetralogy of Fallot, right aortic arch, and malposition of the branch pulmonary arteries. Cardiac angiography confirmed intracardiac anomalies and showed the ostium of the left pulmonary artery lying superiorly and to the right of the right pulmonary artery and their crisscross.

Transcatheter device closure of a residual postmyocardial infarction ventricular septal defect.

Postmyocardial infarction ventricular septal defect (VSD) carries a high mortality and, even after successful surgery, residual defect is common. A 75-year-old woman was admitted with the diagnosis of hyperacute anterior myocardial infarction. Primary percutaneous intervention was performed by stenting of a totally obstructed segment in the proximal left anterior descending artery. The patient's condition deteriorated on the second postprocedural day with a 3/6 pansystolic murmur at the mesocardium. Echocardiography revealed an apical anteroseptal VSD and moderate pulmonary hypertension. She underwent surgical VSD closure with a Gore-Tex patch and coronary artery bypass grafting to the left anterior descending and circumflex arteries. The patient's condition continued to be unstable due to septicemia and hemodynamically significant residual VSD. After medical management of septicemia, the residual defect was successfully closed using a 10-mm Cardio-O-Fix septal occluder under fluoroscopic and transesophageal echocardiographic guidance. The clinical condition of the patient was then stabilized and there was no significant residual shunt on echocardiography on the third postprocedural day.

Atrial septal stenting to increase interatrial shunting in cyanotic congenital heart diseases: a report of two cases.

Aiming to increase mixing at the atrial level, atrial septal stenting was performed in two pediatric cases with cyanotic congenital cardiac diseases. The first case was a 3-month-old male infant with transposition of the great arteries. The second case was an 18-month-old male infant with increased central venous pressure due to postoperative right ventricular outflow tract obstruction. Premounted bare stents of 8 mm in diameter were used in both cases. The length of the stent was 20 mm in the first case and 30 mm in the latter. The procedure was accomplished without any complications. In the first case, oxygen saturation increased approximately 20-25% with no significant interatrial gradient. In the latter, central venous pressure decreased from 16 to 8 mmHg immediately after the procedure. The patient was weaned from the ventilator on the second day and discharged from intensive care unit on the fifth day. Follow-up echocardiograms of both patients showed patent stents with good position relative to the atrial septum. Stenting of the atrial septum seems to be a safe and effective method to create a reliable, nonrestrictive interatrial communication.

The effect of low-carbohydrate diet on left ventricular diastolic function in obese children.

BACKGROUND: This study was conducted to evaluate left ventricle (LV) functions using conventional and tissue Doppler imaging in childhood obesity and to identify the effects of diet on LV diastolic functions. METHODS: Conventional and tissue Doppler echocardiographic measurements were compared in 34 obese children and 24 age- and gender-matched lean controls. Fasting plasma glucose, insulin and homeostatic model assessment of insulin resistance levels were also obtained. Thirty-one of the obese children were subjected to a low-carbohydrate diet and their follow-up measurements were obtained after 6 months. RESULTS: Left atrial diameter, LV mass and LV mass index were higher in obese children than in lean controls. Lateral mitral myocardial early diastolic (E(m)) and peak E(m)/myocardial late diastolic (A(m)) were lower, and mitral E/E(m) and lateral mitral myocardial isovolumetric relaxation time were higher in obese subjects than in lean controls. Insulin and homeostatic model assessment of insulin resistance levels were higher in obese patients and decreased significantly after diet. After diet therapy, lateral mitral E(m) and peak E(m)/A(m), were increased, mitral E/E(m) and myocardial isovolumetric relaxation time were decreased. CONCLUSIONS: Obesity predisposes children to increased preload reserve, left ventricular subclinical diastolic dysfunction and deterioration in diastolic filling. Weight reduction with a low-carbohydrate diet seems to be associated with significant improvement in LV diastolic function and a decrease in diastolic filling, as well as causing reversal in insulin resistance seen in obese children.

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.

Cardiac functions in children with coeliac disease during follow-up: insights from tissue Doppler imaging.

BACKGROUND: The identification of a coeliac disease in patients with idiopathic dilated cardiomyopathy raises critical questions on the relationship between the two entities. But cardiac functions have not been studied in patients with coeliac disease. The present study was undertaken to assess cardiac functions by Tissue Doppler Echocardiography in patient with coeliac disease. METHODS: We studied 45 clinically stable patients; twenty-five patients with positive serum IgA Antiendomysial Antibody levels (Group 1), twenty patients with negative serum IgA Antiendomysial Antibody levels (Group 2) at the time of echocardiographic study. Control group consisted of 30 healthy children free of any disease. RESULTS: Myocardial systolic wave velocity of the mitral annulus was significantly lower (p<0.001), myocardial precontraction and contraction time were slightly longer in Group 2 when compared control group (p=0.015, p=0.044, respectively). There was a negative correlation between the serum IgA Antiendomysial Antibody levels titers and myocardial systolic wave levels of all patients included in the study (r = -0.633; p<0.001). A myocardial systolic wave velocity of <8.9 cm/s had a 92% sensitivity and 80% specificity in predicting serum IgA Antiendomysial Antibody levels positive patients. CONCLUSIONS: We detected subclinical systolic dysfunction of the left ventricle in children with coeliac disease in whom serum IgA Antiendomysial Antibody reactivity is prominent. Tissue Doppler echocardiography provides a quantifiable indicator useful for cardiac monitoring of disease during follow up.

Transaortic and transmitral extended myectomy and concomitant supracoronary myotomy in a girl with hypertrophic cardiomyopathy.

Concomitant idiopathic hypertrophic subaortic stenosis and disseminated myocardial bridging is an uncommon clinical entity with poor prognosis. We describe a symptomatic 19-year-old girl who had myocardial debridging and transaortic and transmitral extended septal myectomy in the same surgical session. An early and simultaneous surgical approach may prevent sudden cardiac death in these high-risk patients.

[Coexistence of partial anomalous pulmonary venous drainage and pulmonary atresia with ventricular septal defect: a report of two cases]. / Ventriküler septal defektli pulmoner atreziye eslik eden kismi pulmoner venöz dönüs anomalisi: Iki olgu sunumu.

Coexistence of partial anomalous pulmonary venous drainage (PVD) and pulmonary atresia with ventricular septal defect (PA-VSD) is very rare with the exception of heterotaxy syndromes. We presented two cases of PA-VSD in which partial anomalous PVD was detected during pulmonary artery angiography. One was a six-month-old girl who underwent cardiac catheterization to evaluate the chance of unifocalization. There were true pulmonary arteries and three major aortopulmonary collaterals. It was noted that the right upper pulmonary vein was in direct continuity with the right atrium. The second case was a 12-year-old girl who was previously diagnosed as having PA-VSD and absence of true pulmonary arteries, for which she had undergone two subsequent aortopulmonary shunt operations four years before. During cardiac catheterization, confluent but hypoplastic true pulmonary arteries and multiple minor aortopulmonary collaterals were detected. The right and left upper pulmonary veins were draining to the right atrium. It should be known that, albeit very rare, partial anomalous PVD may be present in PA-VSD cases, requiring careful evaluation of pulmonary venous drainage during echocardiographic and angiographic studies.

Successful transcatheter balloon dilatation of coarctation of aorta and coil occlusion of patent ductus arteriosus in a single catheterization procedure.

Simultaneous transcatheter coil occlusion of patent ductus arteriosus and balloon angioplasty of coarctation of aorta is avoided since intimal tearing after angioplasty is believed to increase the risk for dissection of the aorta during catheter manipulation. We report a 14-month-old boy, with weight of 7.6 kg, with aortic coarctation and patent ductus arteriosus with a large left-to-right shunt who underwent successful simultaneous balloon angioplasty of native coarctation and coil embolization of the patent ductus arteriosus. Coarctation gradient decreased from 30 to 4 mmHg after balloon angioplasty and complete occlusion of the ductus arteriosus was achieved without any complication.

Stent implantation into the patent ductus arteriosus in cyanotic congenital heart disease with duct-dependent or diminished pulmonary circulation.

We assessed the efficacy and safety of stent implantation into the ductus arteriosus in infants with cyanotic heart disease. Ten patients with duct-dependent or diminished pulmonary circulation underwent cardiac catheterization with the aim of stent implantation. Coronary stents of 3.5-4 mm in diameter were used and successfully implanted in eight. All patients in whom stent implantation was successful had adequate relief of their cyanosis and were discharged home. During a mean follow-up of 9.3 months (median 10 months), one patient had evidence of restenosis of the stent at five months. Re-dilatation of the stenosed stent was attempted but was unsuccessful and a modified left Blalock-Taussig shunt was placed surgically. Stent implantation of ductus arteriosus in neonates and infants with a duct-dependent pulmonary circulation is an effective and safe palliative procedure. It may be an alternative to surgical systemic-pulmonary artery shunt in a select group of patients.

ALPK3 gene mutation in a patient with congenital cardiomyopathy and dysmorphic features.

Primary cardiomyopathy is one of the most common inherited cardiac diseases and harbors significant phenotypic and genetic heterogeneity. Because of this, genetic testing has become standard in treatment of this disease group. Indeed, in recent years, next-generation DNA sequencing has found broad applications in medicine, both as a routine diagnostic tool for genetic disorders and as a high-throughput discovery tool for identifying novel disease-causing genes. We describe a male infant with primary dilated cardiomyopathy who was diagnosed using intrauterine echocardiography and found to progress to hypertrophic cardiomyopathy after birth. This proband was born to a nonconsanguineous family with a past history of a male fetus that died because of cardiac abnormalities at 30 wk of gestation. Using whole-exome sequencing, a novel homozygous frameshift mutation (c.2018delC; p.Gln675SerfsX30) in ALPK3 was identified and confirmed with Sanger sequencing. Heterozygous family members were normal with echocardiographic examination. To date, only two studies have reported homozygous pathogenic variants of ALPK3, with a total of seven affected individuals with cardiomyopathy from four unrelated consanguineous families. We include a discussion of the patient's phenotypic features and a review of relevant literature findings.

Intermediate-term results of Medtronic freestyle valve for right ventricular outflow tract reconstruction in the Ross procedure.

BACKGROUND AND AIM OF THE STUDY: The Ross procedure has become the first choice for aortic valve replacement in children and young adults at many institutions. Since 1997, a lack of availability of homograft valves in Turkey has prompted the use of alternative substitutes for right ventricular outflow tract (RVOT) reconstruction during the Ross procedure. METHODS: Before April 2005, among 20 patients (age range: 14 months to 45 years) at the present authors' institution, the Ross procedure was performed in 14 and a Ross-Konno procedure in six. Sixteen patients underwent RVOT repair using alternative methods for homograft valve replacement. Fourteen patients received a Medtronic Freestyle valve and one patient a Medtronic Contegra bovine jugular vein conduit. An autologous RVOT repair was used in one patient. Ten of the Medtronic Freestyle valve patients were aged <16 years. In all patients who received a Medtronic Freestyle valve echocardiographic evaluations were conducted shortly after surgery and during follow up. RESULTS: There was no early mortality. One patient died from pneumonia after six months, and another (asymptomatic) patient died suddenly at 34 months after surgery. Before hospital discharge the mean peak pressure gradient across the Freestyle valve was 12.1 +/- 11.0 mmHg, and this increased to 24.1 +/- 20.0 mmHg after a mean follow up of 51.2 +/- 6.9 months (range: 6 to 101 months) (p <0.002). Mild pulmonary regurgitation was seen in two patients. One asymptomatic adult patient was reoperated on at another center because of a 60-mmHg echocardiographic peak gradient at four years postoperatively. CONCLUSION: Although long-term follow up is required to explain the durability of the Medtronic Freestyle valve, the present results show that the valve can be used with intermediate-term success in the Ross procedure - and even in children as an alternative - if homograft valves are not available.

Embolization of a complex pulmonary arteriovenous fistula and coarctation treatment with covered stent at the same session.

Pulmonary arteriovenous fistula (PAVF) are rare malformations that may cause serious complications such as paradoxical embolism, stroke, pulmonary hemorrhage and hemoptysis. Accompanying cardiac malformations such as aortic coarctation were not reported previously. Here we present a case of complex PAVF associated with aortic coarctation. The patient was treated successfully by transcatheter embolization of PAVF with amplatzer vascular plug I and II and implantation of a covered CP stent for coarctation at the same session.

Assessment of the anomalous pulmonary circulation by dynamic contrast-enhanced MR angiography in under four seconds.

The purpose of this study was to demonstrate the initial results of the dynamic contrast-enhanced MRA in under four seconds in the assessment of anomalous pulmonary circulation. We performed dynamic contrast enhanced MRA on ten patients with a temporal resolution of 2-3 sec for each phase. Five patients had pulmonary vascular anomaly and five patients had reconstructed pulmonary circulation. On nine patients catheter angiography was performed for the correlation. Dynamic contrast-enhanced MRA successfully demonstrated congenital pulmonary vascular anomalies and the pathway of anomalous pulmonary circulation in our series. With the improved temporal and spatial resolution, the dynamic contrast-enhanced MRA can be used in children and infants for the assessment of congenital vascular disease.

A case of tuberous sclerosis presenting with dysrhythmia in the first day of life.

Cardiac rhabdomyoma (CR) is the most common primary cardiac tumor in childhood. Although CRs are asymptomatic in many cases, they may cause arrhythmia, heart failure and fetal hydrops. Babies with arrhythmia in the neonatal period must be investigated for structural heart disease including CR. Cardiac rhabdomyoma may either present as an isolated tumor or may be related with tuberous sclerosis. Arrhythmia due to CR may be the initial sign of tuberous sclerosis. We report a case of tuberous sclerosis presenting with ventricular premature beats and second-degree atrioventricular block in the first day of life who was found to have multiple CR during echocardiographic examination.