Right pulmonary artery to left atrium fistula.

In this report, we describe a 25-year-old patient in whom a fistula between the right pulmonary artery and the left atrium was accurately diagnosed. The successful surgical management of this patient is described. Current diagnostic methods, evaluation, and treatment options for this uncommon cause of cyanosis in an adult patient are discussed.

Totally anomalous pulmonary venous connection with intact atrial septum.

BACKGROUND: Total anomalous pulmonary venous connection with intact interatrial septum is extremely rare. AIMS: In these patients, a right to left shunt through a ventricular septal defect or a patent ductus arteriosus is mandatory to maintain the systemic circulation. Mechanical or physiological restriction of shunting through these pathways results in rapid clinical deterioration. DISCUSSION: We describe the anatomical findings with surgical repair in one such 10-day-old baby.

The use of an autologous right atrial free wall as a patch for closure of atrial septal defects.

BACKGROUND: Various patch materials to close large atrial septal defects (ASDs) are autologous pericardium or a large of prosthetic patches which may be associated with infrequent but definite problems. We describe our experience with the use of the right atrial free wall patch over the last two decades. METHODS AND RESULTS: Between July 1998 and December 2017, 157 patients (mean age 14.7 ± 13.9 years), underwent ASD closure using the right atrial free wall patch. Associated lesions were severe mitral regurgitation (n = 24), partial anomalous pulmonary venous drainage (n = 15), ASD closure occurring after myxoma excision (n = 12) total anomalous pulmonary venous drainage (n = 2) and tricuspid regurgitation (n = 2). Surgery was uneventful in all patients. All patients underwent serial electrocardiography and echocardiography. Follow-up in 140 of 155 survivors was 103.6 ± 0.6 months. One hundred forty of one hundred fifty-five survivors are in sinus rhythm and three have persistent atrial fibrillation. Twenty-four hours of Holter monitoring (n = 19) revealed normal sinus rhythm in all but three patients, with occasional atrial ectopics in one patient; four were lost to follow-op. Electrophysiological studies in seven consenting patients, 9 to 16 months following ASD closure showed normal atrial potentials from the site of the patch. CONCLUSIONS: The autologous right atrial free wall is a safe patch material for ASD closure. Its advantages are that it is autologous, endothelialized, probably viable, and carries a low risk of thromboembolism. Studies with a larger number of patients with longer follow are needed to further confirm these findings.

Comparison of pleural effusion between fenestrated and nonfenestrated extracardiac Fontan: A prospective randomized study.

BACKGROUND: Fenestration of the baffle/conduit is believed to reduce pleural effusion following the Fontan operation. However, equivocal results have been observed with or without fenestration. This study aims to evaluate the efficacy of fenestration on the amount and duration of pleural effusion following the Fontan operation. METHODS: About 40 patients undergoing extracardiac Fontan (ECF) were randomized into two groups: one with fenestration (ECF-F; n = 20) or without fenestration (ECF-NF; n = 20). Primary outcome was the amount and duration of pleural effusions. Secondary outcomes were time to removal of the chest tubes, hospital stay, and readmission to the hospital because of recurrent pleural within 30 days of the operation. RESULTS: Mean age was 11.5 ± 5.07 (range, 8.7-13.5) years in the ECF-F group and 13.6 ± 0.4 years (range, 10.5-15.5) in the (ECF-NF) group. The total drain output was 7.89 mL/kg/d in ECF-NF compared with 6.9 mL/kg/d in the ECF-group (P = .14). Time for removal of pleural tubes was 14.6 ± 0.95 days in the ECF-NF group compared with 11.6 ± days in the ECF-F group. Total duration of hospital stay was higher but not significant in the ECF-NF group compared with the ECF-F group. Two patients in ECF-NF required readmission to the hospital within 30 days following discharge, while there were no readmissions in the ECF-F group. CONCLUSION: Contrary to the literature, the creation of a fenestration in the ECF circuit was not clearly associated with a reduction in the amount and duration of pleural effusion compared with a non-fenestrated Fontan. These findings may be debatable in high risks versus low risk candidates. However in the present study, in a low risk canditates undergoing the Fontan operation, the daily amount of pleural drainage was no different. Larger studies are needed to confirm these findings.

Ruptured sinus of valsalva presenting late following repair of tetralogy of fallot.

Rupture of sinus of valsalva following repair of tetralogy of fallot (TOF) is very rare. It should be suspected as a cause of recurrent or prolonged pleural effusion and congestive cardiac failure in patients who have undergone repair of TOF. We report one such patient.

Unusual presentation and rupture of left sinus of Valsalva into mitral-aortic intervalvular fibrosa.

Ruptured sinus of Valsalva aneurysm from the left coronary sinus communicating through the mitral-aortic intervalvular fibrosa is rare. We report a 35-year-old patient who presented with only hemoptysis without any other cardiac complaint. The patient with this unusual diagnosis and presentation was managed successfully.

First Report of Stem Rot Caused by Athelia rolfsii on Curry Leaf Tree (Murraya koenigii) in Tripura, India.

Murraya koenigii is an important medicinal plant of India and commonly known as curry leaf tree grown in tropical and subtropical regions. The leaves of curry tree are used as a herb due to the presence of following important active constituent bismahanine, murrayanine, murrayafoline-A, bi-koeniquinone-A, murrayazolidine etc. (Jain et al. 2017). During mid-July 2019, stem rot disease symptoms were observed on curry leaf trees at the College of Agriculture, Lembucherra, Tripura (India). The disease symptoms consisted of rotting, wilting and blighting with disease incidence ranging from 8 to 10%. Initially, infected plants gradually withered and white mycelia mats appeared on the surface of the lower stem at the soil line. Infected stem samples were collected and surface was sterilized with 0.25% sodium hypochlorite for 1 min, washed thrice with sterilized distilled water and placed in Petri plates containing 2% water agar. After three days of incubation at 26°C, hyphae produced from plant bits were transferred into Petri plates containing potato dextrose agar. Ten isolates were collected from the diseases samples. Pure cultures were obtained as abundant, aerial and white mycelia with round to irregular sclerotia of 0.8 to 1.5 mm in diam. The sclerotia were initially white in color but later turned into brown color. The pathogen was identified as Athelia rolfsii based on morphology (Aycock 1966). To confirm the identification, the genomic DNA was extracted from a mycelia mat of the isolates using ZR fungal/Bacterial DNA miniprep kit (Irvine, CA) and the internal transcribed spacer (ITS) region of rDNA was amplified using the universal primers, ITS1 and ITS4 (White et al. 1990). A 550 bp PCR product was sequenced and showed 99% similarity with Athelia rolfsii isolate (GenBank accession MH854711).The generated sequence was submitted to GenBank (Accession MT535585). After identification of the pathogen a pot experiment was conducted to confirm the pathogenicity. Earthen pots (29 cm. diam.) were filled with sterilized soil and kept in a green house. Ten curry leaf plants (50 days old) were grown from seed in the separate pot were inoculated with 15-day old mycelia mats prepared in potato dextrose broth. The stem of each curry plant were artificially injured with the help of sterile blade and wrapped with moistened sterilized cotton containing the mycelial mat. Five curry leaf plants artificially injured and inoculated with sterilized distilled water were used as control. The Earthen pots with plants were individually covered with plastic bags and kept in the green house at 26°C for approximately 15 days. The inoculated plants started showing symptoms of stem rot six days after inoculation and started drying onward. The symptoms of stem rot on the inoculated plants were similar to those observed in the field. The fungus was re-isolated from the inoculated plants and A. rolfsii identification was confirmed based on morphology. No symptoms were observed on the control plants. The obtained culture was deposited in the Indian Type Culture Collection, Division of Plant Pathology, ICAR - Indian Agricultural Research Institute, New Delhi, India (ITC-8666). To the best of our knowledge this is the first report of stem rot disease of curry leaf plant caused by A. rolfsii in India and worldwide. Due to medicinal, flavour and aroma properties, it is regularly used in India. Curry leaf plant is regularly used as a medical herb in India and therefore this disease poses a significant risk to production.

Surgical repair for aortopulmonary window with interrupted aortic arch in late childhood.

Aortopulmonary window with interrupted aortic arch is rarely reported beyond infancy. Pre-operative assessment and surgical repair are challenging. We report successful surgical repair of aortopulmonary window with interrupted aortic arch in a 6-year-old girl with near-normal pulmonary artery pressure immediately following surgery.

An alternative technique for completion of the total cavopulmonary connection.

BACKGROUND: Total Cavopulmonary connection (Fontan) is the final palliation for patients with a functionally univentricular heart. This is commonly accomplished after a prior bidirectional Glenn on cardiopulmonary bypass (CPB) with separate cannulation of the aorta, superior vena cava (SVC), and inferior vena cava. We describe an alternative technique of Fontan completion that eliminates the need for cannulation and dissection of the SVC, and pulmonary artery dissection. METHODS: Between January and October 2018, 17 patients underwent completion Fontan using an alternate technique at our institute. All operations were conducted on CPB at normothermia without cannulating the SVC RESULTS: Mean CPB time was 60 ± 16.8 minutes (range, 39-102 minutes). There were no early deaths. Mean postoperative Fontan pressures were 15.6 ± 1.2 mm Hg with no gradient between the SVC and IVC pressures. Mean duration of hospital stay was 15.6 ± 3.6 days (range, 10-22 days). No patient developed phrenic nerve paresis or palsy. CONCLUSIONS: Completion without cannulating the SVC is simple, reproducible, and easy to teach. It avoids the disadvantages associated with routine techniques.

An alternative technique for intracardiac exposure during transatrial repair of tetralogy of fallot.

The commonly used technique to facilitate intracardiac exposure during transatrial repair of tetralogy of fallot involves considerable retraction of the tricuspid valve using retractors. We describe an alternative surgical technique in which it is possible to dispense away with the retractors. The advantages of such a technique are discussed.

Unilateral unidirectional superior cavopulmonary anastomosis in a patient with bilateral superior caval veins and atretic left pulmonary artery.

A unidirectional superior cavopulmonary anastomosis was performed on the right side in a patient with a functionally univentricular heart, atresia of main and left pulmonary artery, bilateral superior caval veins, and a patent arterial duct in the right pulmonary artery. Anastomosis of the left superior caval vein to the right superior caval vein created a neo-innominate vein without using prosthetic material.

Multiple saccular aortic aneurysms following the arterial switch operation.

We report a 3-month-old male presenting with multiple aortic aneurysms arising de novo 2 months following the arterial switch operation. Successful repair of the aneurysms was performed under total circulatory arrest and at seven years follow-up, the patient has no recurrence.

Ruptured sinus of Valsalva aneurysm with tetralogy of Fallot in an adult.

A combination of ruptured sinus of Valsalva (RSOV) aneurysm with tetralogy of Fallot (TOF) is a very rare condition. We report a 32-year-old male who underwent repair of a RSOV aneurysm with TOF.

Bidirectional Glenn on cardiopulmonary bypass: A comparison of three techniques.

OBJECTIVE: To analyze the intraoperative and early results of the bidirectional Glenn (BDG) procedure performed on cardiopulmonary bypass (CPB) using three different techniques. METHODS: Between September 2013 and June 2015, 75 consecutive patients (mean age 42 ± 34.4 months) undergoing BDG were randomly assigned to either technique I: open anastomosis or technique II: superior vena cava (SVC) cannulation or technique III: intermittent SVC clamping. We monitored the cerebral near infrared spectrophotometry (NIRS), SVC pressure, CPB time, intensive care unit (ICU) stay, and neurocognitive function. RESULTS: Patients in technique III had abnormal lower NIRS values during the procedure (57 ± 7.4) compared to techniques I and II (64 ± 7.5 and 61 ± 8.0, P = 0.01). Postoperative SVC pressure in technique III was higher than other two groups (17.6 ± 3.7 mmHg vs. 14.2 ± 3.5 mmHg and 15.3 ± 2.0 mmHg in techniques I and II, respectively = 0.0008). CPB time was highest in technique II (44 ± 18 min) compared to techniques I and III (29 ± 14 min and 38 ± 16 min, P = 0.006), respectively. ICU stay was longer in technique III (30 ± 15 h) compared to the other two techniques (22 ± 8.5 h and 27 ± 8.3 h in techniques I and II, respectively = 0.04). No patient experienced significant neurocognitive dysfunction. CONCLUSION: All techniques of BDG provided acceptable results. The open technique was faster and its use in smaller children merits consideration. The technique of intermittent clamping should be used as a last resort.

Bidirectional superior cavopulmonary anastomosis with or without cardiopulmonary bypass: A randomized study.

OBJECTIVES: This study aims to compare the bidirectional superior cavopulmonary anastomosis (BDG) with or without cardiopulmonary bypass (CPB). METHODS: 100 patients undergoing BDG were randomized into two groups: Off-CPB or on-CPB groups. All patients underwent near-infrared spectrophotometry (NIRS) and bispectral index (BIS) monitoring and pre- and postoperative serum 100 beta protein measurements (Sß100) and neuro-cognitive evaluation. Postoperative intensive care unit (ICU) parameters were also studied. RESULTS: The median age of patients in the on-CPB and off-CPB group were 42 and 48 months, respectively (p = 0.11). Median weights in the on-CPB group and off-CPB group were 13.5 (5-50) kg and 15 (7-36) kg, respectively (p = 0.927). There was a significant rise in superior vena cava (SVC) pressure on SVC clamping in the off-CPB group (23.12 ± 6.84 vs 2.98 ± 2.22 mmHg) on-CPB group (p < 0.001). There was a significant fall in NIRS and BIS values from baseline in the off-CPB group during the anastomosis but there was no statistically significant change in serum Sß100from pre-clamp to post-clamp in either group. Inotropic support, duration of ventilation, ICU stay, and hospital stay were significantly less in the off-CPB group (p < 0.001). Assessment of Social Adaptive Functioning revealed no adverse sequelae. There were significant cost savings if surgery was performed off-CPB (p < 0.001). CONCLUSION: Off CPB-BDG is an economical and safe procedure. Duration of inotropic and mechanical ventilatory support, ICU, and hospital stay is significantly less. We did not observe any early adverse neurologic sequelae in patients undergoing off-CPB BDG.

Aortopulmonary window: Morphology, diagnosis, and long-term results.

OBJECTIVE: Aortopulmonary window (APW) is a rare congenital heart defect. We reviewed our experience with this condition over the last two decades. METHODS: Between September 1993 and December 2013, 62 patients underwent surgery for APW. Depending on the associated lesions, they were divided into two groups: Simple (Group 1) or complex (Group 2). In the complex group, six patients had a ventricular septal defect, five patients had interrupted aortic arch, three patients had tetralogy of Fallot, two patients had double outlet right ventricle, and one patient had the right pulmonary artery arising from the ascending aorta. RESULTS: Mean age at repair was 21.6 ± 32.02 months (median = 6, range 0.1-144 months). By preoperative echocardiographic assessment 27 out of 62 patients had severe pulmonary artery hypertension (52% of the cohort). Patch repair of APW was performed using the sandwich method (transwindow) (n = 27; 43.5%), transaortic (n = 18; 29%), and transpulmonary artery (n = 5; 8.1%) approaches; 10 patients (16.1%) underwent double ligation and two (3.2%) underwent division and suturing. Overall hospital mortality in group 1 was 6.97% (3/43) and in group 2 it was 21% (4/19), p = 0.085. Mean hospital stay in group 1 was 6.9 ± 2.4 days (median = 7 days) and in group 2 was 12 ± 6.1 days (median = 13 days), p = 0.0001. Follow-up in group 1 was 1.6-9.8 years (median = 6 years); in group 2, it was 1.8-8.9 years (median = 6.5 years). There were no late deaths. Two patients needed reintervention for distortion of the right pulmonary artery origin. All patients were in New York Heart Association Class I/II at last follow up. CONCLUSION: There are multiple acceptable surgical strategies for the treatment of aortopulmonary window. Despite a relatively advanced age and substantial number of patients with severe pulmonary hypertension the outcomes can still be good. Associated anomalies complicate the repair. Patients in the complex group had a protracted hospital course and a higher early mortality but similar late survival.

Resection of subaortic membrane for discrete subaortic stenosis.

BACKGROUND: We reviewed the long-term results of surgery for discrete subaortic membrane (SubAM) from a single institute. METHODS: A retrospective review of medical records of all patients (n = 146) who underwent resection of a SubAM for discrete subaortic stenosis between 1990 and 2015 at the All India Institute of Medical Sciences, New Delhi, India was undertaken. RESULTS: Median age at surgery was 9.0 years (9 months-47 years). There was one early death. Preoperative peak left ventricular outflow tract (LVOT) Doppler gradient was 83.4 ± 26.2 mmHg (range: 34-169 mmHg). On preoperative echocardiography, aortic regurgitation (AR) was absent in 69 (47.3%), mild in 35 (24%), moderate in 30 (20.5%), and severe in 12 (8.2%). After surgery, the LVOT gradient was reduced to 15.1 ± 6.2 mmHg (P < 0.001). Fourteen patients (9.6%) who had residual/recurrent significant gradients are currently being followed-up or awaiting surgery. There was improvement in AR for operated patients with freedom from AR of 92.6 ± 0.03% at 15 years. Kaplan-Meier survival at 25 years was 93.0 ± 3.9% (95% confidence interval: 79.6, 97.7). Freedom from re-operation at 25 years was 96.9 ± 1.8%. CONCLUSIONS: Long-term results of surgery for discrete SubAM are good. Resection of the membrane along with septal myectomy decreases the risk of recurrence.

Arterial switch operation in patients with transposition and a left-sided aorta.

OBJECTIVES: Arterial switch operation is the treatment of choice in infants with transposed arterial trunks. It is technically challenging to perform in patients having usual atrial arrangement and concordant atrioventricular connections but having a left-sided aorta. Correction in this setting requires surgical expertise and precision. Here we review our experience with such patients. METHODS: Between January, 2002 and October, 2013, the arterial switch operation was performed in 20 patients in the combination emphasised above. Patient records were analysed in detail for coronary arterial patterns, and for the techniques used for transfer of the coronary arteries and reconstruction of the great arteries. Outcomes were recorded in terms of in-hospital survival and left ventricular function at the most recent follow-up. RESULTS: All patients survived the procedure. Ages ranged from 3 days to 18 months, with a median of 75 days; the weight of the patients ranged from 3 to 8.8 kg, with a median of 3.85 kg. The LeCompte manoeuvre was performed in only nine patients. The mean cardiopulmonary bypass time was 157.5±24.9, with a median of 161 minutes, and the mean aortic cross-clamp time was 101.2±23.8, with a median of 102 minutes. Subsequently, two patients died: the first due to a sudden onset of ventricular fibrillation and the second during a crisis of severe pulmonary hypertension. At the last follow-up, which ranged from 23 to 41 months, with a mean of 38.04±2.32 and a median of 38.4 months, all 18 survivors were in NYHA class I, with none requiring cardiac medications and all having normal bi-ventricular function without residual defects. CONCLUSION: With appropriate technical modifications, patients with concordant atrioventricular and discordant ventriculo-arterial connections with a left-sided aorta can undergo successful anatomical repair.