Valley Index as a Predictor of Prenatal Diagnosis of Total Anomalous Pulmonary Venous Connection.

INTRODUCTION: Total anomalous pulmonary venous connection (TAPVC) has a low prenatal diagnostic rate. Therefore, we investigated whether Doppler waveforms with a low pulsatility in the pulmonary veins can indicate fetal TAPVC. METHODS: This retrospective study included 16 fetuses with TAPVC, including 10 with complex congenital heart disease and 104 healthy fetuses that underwent fetal echocardiography. Pulmonary venous S and D wave flow velocities and the valley (representing the lowest velocity between the S and D waves) were measured. Valley indices I and II were then calculated as (velocity of valley/greater of the S and D wave velocities) and (velocity of valley/lesser of the S and D wave velocities), respectively. RESULTS: Supra/infracardiac TAPVC cases exhibited significantly greater valley indices than that of the healthy group. After adjusting for gestational age at fetal echocardiography, valley indices I (odds ratio [OR] 7.26, p < 0.01) and II (OR: 9.23, p < 0.01) were significant predictors of supra/infracardiac TAPVC. Furthermore, valley indices I and II exhibited a high area under the curve for detecting supra/infracardiac TAPVC, regardless of the presence of pulmonary venous obstruction. CONCLUSION: The valley index may be a useful tool for the detection of fetal TAPVC.

External validation of the rCAST for patients after in-hospital cardiac arrest: a multicenter retrospective observational study.

No established predictive or risk classification tool exists for the neurological outcomes of post-cardiac arrest syndrome (PCAS) in patients with in-hospital cardiac arrest (IHCA). This study aimed to investigate whether the revised post-cardiac arrest syndrome for therapeutic hypothermia score (rCAST), which was developed to estimate the prognosis of PCAS patients with out-of-hospital cardiac arrest (OHCA), was applicable to patients with IHCA. A retrospective, multicenter observational study of 140 consecutive adult IHCA patients admitted to three intensive care units. The area under the receiver operating characteristic curves (AUCs) of the rCAST for poor neurological outcome and mortality at 30 days were 0.88 (0.82-0.93) and 0.83 (0.76-0.89), respectively. The sensitivity and specificity of the risk classification according to rCAST for poor neurological outcomes were 0.90 (0.83-0.96) and 0.67 (0.55-0.79) for the low, 0.63 (0.54-0.74) and 0.67 (0.55-0.79) for the moderate, and 0.27 (0.17-0.37) and 1.00 (1.00-1.00) for the high-severity grades. All 22 patients classified with a high-severity grade showed poor neurological outcomes. The rCAST showed excellent predictive accuracy for neurological prognosis in patients with PCAS after IHCA. The rCAST may be useful as a risk classification tool for PCAS after IHCA.

Cilostazol treats transient heart failure caused by ATP1A3 variant-associated polymicrogyria.

BACKGROUND: Some patients with ATP1A3 variant-associated polymicrogyria have recurrent transient heart failure. However, effective treatment for the transient cardiac condition remains to be elucidated. CASE REPORT: The patient started experiencing focal motor onset seizures in 12 h after birth, revealing bilateral diffuse polymicrogyria. The patient also experienced transient bradycardia (sinus bradycardia) attacks from 15 days old. Echocardiography revealed a reduced ejection fraction; however, no obvious electrocorticogram or electroencephalogram abnormalities were observed during the attacks. Initially, the attacks occurred in clusters daily. They later decreased in frequency, occurring at monthly intervals. Repeated episodes of transient bradycardia attacks and polymicrogyria indicated possible ATP1A3 gene abnormality and genetic testing revealed a novel heterozygous ATP1A3 variant (NM_152296: exon22:c.2977_2982del:p.(Glu993_Ile994del)), which was not found in the patient's parents. Cilostazol was administered at 3 months old for recurrent transient bradycardia attacks. Cilostazol significantly shortened the duration of bradycardia episodes and prolonged the interval between attacks. Cilostazol also effectively treats transient symptomatic bradycardia. CONCLUSION: Cilostazol could be a treatment option for recurrent transient bradycardia attacks associated with ATP1A3 gene abnormalities and polymicrogyria.

Successful perioperative management of pheochromocytoma in a patient with Fontan circulation.

Pheochromocytoma (PCC) can adversely affect Fontan circulation. However, there are few reports on its perioperative management before and after PCC resection in Fontan patients. A 24-year-old female patient with congenitally corrected transposition of the great arteries, ventricular septal defect, and pulmonary atresia who had undergone Fontan palliation developed heart failure caused by PCC. The patient was pre-conditioned for PCC resection with heart failure treatment, alpha-blocker titration, and careful infusion, and had a good intraoperative and postoperative course with no complications. Postoperative catheter data showed improvements in systemic vascular resistance, cardiac output, and central venous pressure compared with preoperative data. There is no established preconditioning method for PCC resection in patients with Fontan circulation. Careful perioperative management based on an understanding of the features of the Fontan circulation can lead to better outcomes. Learning objective: Pheochromocytoma (PCC) can occur in patients with Fontan circulation. Preoperative management and the PCC itself can adversely affect Fontan circulation, highlighting the importance of suspecting PCCs in Fontan patients based on symptoms such as heart failure, worsening arrhythmias, and headache, and emphasizing careful perioperative management.

Agenesis of the venous duct with abnormal return of the umbilical vein into the superior vena cava: A case report.

INTRODUCTION: Agenesis of the venous duct is rare, with an incidence rate of .04%-.6%. Abnormal drainage of the umbilical vein (UV) to superior vena cava (SVC) is seen in .5% of agenesis of the venous duct cases. We present a case of agenesis of the venous duct with drainage of the UV into the SVC accompanied by tetralogy of Fallot. CASE PRESENTATION: The fetus was diagnosed with agenesis of the venous duct and tetralogy of Fallot at 29 gestational weeks. The UV directly returned to the SVC. Cardiomegaly and pericardial effusion were observed but did not deteriorate. The female infant was born at 40 gestational weeks. Contrast-enhanced computed tomography showed that the UV was occluded at its proximal aspect. No abnormality of the portal system was noted. The infant underwent intracardiac repair and was doing well at 16 months of age. DISCUSSION/CONCLUSION: Although the extrahepatic drainage type of agenesis of the venous duct is occasionally associated with heart failure and hydrops, severe hydrops was absent in this case. It was speculated that vascular resistance in the long pathway to the SVC restricted direct inflow from the UV. Portosystemic shunts and agenesis of the portal system are reported complications of agenesis of the venous duct. Prenatal agenesis of the venous duct diagnosis may be crucial for early postnatal diagnosis of these conditions.

Fetal case of rare association of hypoplastic left heart syndrome and absent atrial septum accompanied by mixed form of supracardiac total anomalous pulmonary venous connection.

Abnormal pulmonary venous flow patterns on fetal echocardiography and a nutmeg lung pattern on fetal magnetic resonance imaging are seen in patients with pulmonary venous stenosis. The association between these findings and the degree of pulmonary venous stenosis remains unknown. We report an extremely rare case of a fetus diagnosed with hypoplastic left heart syndrome complicated by an absent atrial septum and supracardiac total anomalous pulmonary venous connection with left pulmonary venous congestion. This case suggests that compared to non-pulsatile continuous pulmonary venous flow, the nutmeg lung pattern can only be observed with severe pulmonary congestion and advanced pulmonary lymphangiectasia.

Absent pulmonary valve with tricuspid atresia/stenosis: literature review with new three long-term cases.

Absent pulmonary valve (APV) syndrome with tricuspid atresia or tricuspid stenosis (TA/TS) is an extremely rare malformation recently reported as a variant of APV with intact ventricular septum (VS). The condition, however, has univentricular physiology and unique structural and clinical features. The purpose of this study was to update the current knowledge about this condition by describing long-term outcomes of three new cases and reviewing the available literatures. A systematic literature search was performed to collect clinical and anatomical data of APV with TA/TS. Institutional medical records were retrospectively reviewed to identify APV with TA/TS patients. In a total of 62 (59 reported and 3 new) cases, patent ductus arteriosus was present in 98% of APV patients with TA/TS. A large ventricular septal defect, dilatation of the pulmonary arteries, which is typically found in APV with tetralogy of Fallot, and respiratory distress at birth were rarely reported. Most of the recent cases were successfully managed by the Glenn or Fontan procedure. Coronary artery anomaly and ventricular arrhythmia were more frequently reported as the cause of death or severe neurological sequelae (9/16 and 3/8, respectively). Additional surgical intervention was required in the mid/long-term period in three cases due to left-ventricular outflow obstruction and in two due to aortic dilatation. The Fontan and Glenn procedures improved the survival in the last two decades. In addition to coronary artery anomaly and ventricular arrhythmia, left-ventricular outflow tract obstruction and aortic dilatation should be carefully monitored.

A double-barrelled aorta with high aortic Arch.

A double-barrelled aorta was detected in a female newborn with 22q11.2 deletion syndrome. Double-barrelled aorta had been previously described as persistence of the fifth pharyngeal arch, but its existence continues to be debated. Recent embryologic studies suggest that double-barrelled aorta is more likely explained by other developing processes in the majority of cases. In our case, catheter angiography confirmed the presence of the high aortic arch and double-barrelled aorta. The upper lumen was located above the level of the clavicles. These findings suggested that the persistence of the segment of dorsal aorta between the third and fourth embryonic arches and the double-barrelled aorta was more likely a consequence of persistence of the third and fourth pharyngeal arches. Detailed imaging and embryologic considerations played an important role in accurate assessment of the origin of the double-barrelled aorta. .

Prenatally diagnosed left atrial appendage aneurysm with various postnatal imaging investigations: A case report.

A congenital left atrial appendage aneurysm (LAAA) is a rare cardiac malformation, that is, usually diagnosed in adulthood. It is rarely diagnosed prenatally. In most cases, surgical resection is recommended soon after the diagnosis has been made due to the risk of arrhythmia and thrombotic events. The present report describes a case of LAAA that was prenatally diagnosed and was asymptomatic postnatally. Imaging revealed the relation of the cardiac and airway structures around the LAAA in detail. The patient underwent surgical resection of the LAAA successfully at 7 months of age and is currently healthy at 5 years of age.

The Value of Interleukin-6 among Several Inflammatory Markers as a Predictor of Respiratory Failure in COVID-19 Patients.

Patients with coronavirus disease 2019 (COVID-19) develop severe respiratory failure within a short period during the clinical course. It is essential to predict respiratory deterioration in the short term. We investigated the use of inflammatory markers to predict respiratory distress within three days from their analysis in COVID-19 patients. This retrospective observational study included 81 patients admitted with COVID-19. Patients were divided into two groups according to whether the maximum fraction of inspired oxygen (FiO2) for three days from the blood marker measurements was ≥0.4 (high FiO2 group; HFG) or <0.4 (low FiO2 group; LFG). Interleukin-6 (IL-6), C-reactive protein (CRP), lactate dehydrogenase (LDH), white blood cell, D-dimer, and creatinine levels were compared between the two groups. The levels of all markers were significantly higher in HFG patients. Areas under the receiver operating characteristic curve of IL-6, CRP, and LDH had high values of 0.85, 0.82, and 0.81, respectively. The odds ratio of IL-6 which was crude and adjusted for dexamethasone administration initiated before laboratory measurement, showed the high value of 29.1 (5.6-295.6) and 53.9 (4.5-3242.8), respectively. IL-6 can be used as a reliable marker for predicting respiratory illness within three days after assessment.

Successful laparoscopy-assisted repair of a rectovaginal fistula after low anterior resection for rectal cancer: a report of two cases.

BACKGROUND: Rectovaginal fistula (RVF) after low anterior resection for rectal cancer is troublesome and refractory. Although various surgical procedures have been previously described, no definitive procedure has shown a satisfactory outcome. We present two consecutive Japanese patients who underwent successful surgery for an RVF after low anterior resection. CASE PRESENTATION: The patients were two women (61-year-old and a 64-year-old). They were admitted to our hospital with a chief complaint of fecal discharge from the vagina after low anterior resection using the double-stapling technique for rectal cancer. They were diagnosed with RVF. Local surgical procedures, including diverting ileostomy, were unsuccessful in previous hospitals. Therefore, we performed laparoscopy-assisted repair of the RVF. In both patients, laparoscopically robust pelvic adhesions were dissected, and the sigmoid colon was transected at just oral side to the RVF. Thereafter, in combination with a perineal approach, the rectum, along with a previous anastomosis and fistula, were completely removed. Surgeries were completed after vaginal repair, redo coloanal anastomosis, and interposition of the dissected connective tissue. In both patients, the postoperative courses were uneventful. They complained of neither recurrence of any RVF nor fecal incontinence 1 year and 10 months after diverting stoma closure. CONCLUSIONS: A laparoscopy-assisted procedure with reanastomosis and interposition of the perineal connective tissue can be an effective treatment for RVF after low anterior resection for rectal cancer.

Electrocardiographic features of arrhythmogenic right ventricular cardiomyopathy in school-aged children.

It can be difficult to distinguish children with early-stage arrhythmogenic right ventricular cardiomyopathy (ARVC) from those with benign premature ventricular contraction (PVC). We retrospectively evaluated six school-aged children with ARVC and compared with those of 20 with benign PVC. The median age at initial presentation was 11.4 and 10.2 years in ARVC and benign PVC, respectively. None of the ARVC patients fulfilled the diagnostic criteria of ARVC at initial presentation. At ARVC diagnosis, the treadmill exercise test and Holter monitoring showed provoked PVC during exercise and pleomorphic PVC in all ARVC cases, respectively. During the observation period, terminal activation duration (TAD) was prolonged in all ARVC patients. In addition, ΔTAD (5.5 [3-10] ms) were significantly longer than those with benign PVC (p < 0.001). A new notched S-wave in V1 appeared in four (67%) ARVC patients, who had myocardial abnormalities in the right ventricle, and in zero benign PVC. Our electrocardiographic findings, such as provoked PVC during exercise, pleomorphic PVC, prolonged TAD, and a new notched S-wave in V1 could contribute to the early detection of ARVC in school-aged children.

Successful treatment of infantile-onset ACAD9-related cardiomyopathy with a combination of sodium pyruvate, beta-blocker, and coenzyme Q10.

Mitochondrial acyl-CoA dehydrogenase 9 (ACAD9) deficiency is one of the common causes of respiratory chain complex I deficiency, which is characterized by cardiomyopathy, lactic acidemia, and muscle weakness. Infantile cardiomyopathy is the most common phenotype and is usually lethal by the age of 5 years. Riboflavin treatment is known to be effective in ~65% of the patients; however, the remaining are unresponsive to riboflavin and are in need of additional treatment measures. In this report, we describe a patient with ACAD9 deficiency who developed progressive cardiomyopathy at 8 months of age. As the patient's left ventricular ejection fraction (LVEF) kept decreasing to 45.4% at 1 year 8 months, sodium pyruvate treatment was introduced together with a beta-blocker and coenzyme Q10. This resulted in a steady improvement, with full and sustained normalization of cardiac function without riboflavin. The therapy, therefore, might be a useful addition for the treatment of ACAD9 deficiency.

The TNNI3 Arg192His mutation in a 13-year-old girl with left ventricular noncompaction.

Left ventricular noncompaction (LVNC) is a distinct cardiomyopathy that is morphologically characterized by a two-layered myocardium, numerous prominent trabeculations, and deep intertrabecular recesses communicating with the left ventricular cavity. We present a case report regarding the identification of a new mutation in TNNI3 in a patient with LVNC using next-generation sequencing. A 13-year-old girl who had no family history of cardiac disease was hospitalized with dyspnea after exercise and electrocardiographic abnormalities during a school screening. Based on her clinical features, she was diagnosed with LVNC. Via genetic analysis, a TNNI3 heterozygous missense variant was identified in the proband. Although mutations in TNNI3 have been reported in patients with hypertrophic cardiomyopathy and restrictive cardiomyopathy, this is the first report of a mutation in this gene in a patient with LVNC. .

Successful non-operative management of traumatic extrahepatic portal venous injury without intraperitoneal hemorrhage: a case report.

Case: A 52-year-old woman was admitted to our hospital with hypotension after falling from the fifth floor of an apartment building. Contrast-enhanced computed tomography showed liver injury with extravasation of contrast material from the hepatic artery, and extrahepatic portal venous injury with extravasation and pseudoaneurysm. Intra-abdominal hemorrhage was not observed, and bleeding was confined to the retroperitoneal space. Hepatic arteriography showed extravasation, while portal venography showed pseudoaneurysm but no extravasation. After transarterial embolization, the patient's vital signs improved. Non-operative management was selected for the portal venous injury. Outcome: Computed tomography on the 58th hospital day revealed disappearance of the portal venous pseudoaneurysm. The patient was discharged on the 90th hospital day without any complications. Conclusion: This case shows that non-operative management can be selected for portal venous injury when there is no retroperitoneal injury and bleeding is confined to the retroperitoneal space.

[A Case Report of Reoperation for Intractable Obstruction of the Stomach after Surgery for Esophageal Cancer].

Although esophageal reconstruction using the stomach is common in surgery for esophageal cancer, this procedure sometimes results in delayed gastric emptying and reflux. This is a case report of reoperation for intractable obstruction of the stomach after initial esophageal surgery in a 59-year-old man. The obstruction was resistant to conservative management. We resected the duodenum, preserving the vascular pedicle of the right gastroepiploic vessels, and performed reconstruction with a Roux-en-Y procedure in the second operation. There was marked improvement in gastric emptying.

Radiofrequency catheter ablation of idiopathic left anterior fascicular ventricular tachycardia in children.

BACKGROUND: Idiopathic ventricular tachycardia of left anterior fascicular origin (IVT-LAF) is a rare condition, and radiofrequency catheter ablation (RFCA) therapy has not been reported in children. OBJECTIVE: This study aimed to evaluate the procedures and outcomes of RFCA for pediatric IVT-LAF. METHODS: Pediatric IVT-LAF cases for which RFCA was performed between June 2006 and May 2012 at our hospital were reviewed. RESULTS: Of 537 pediatric cases of RFCA, 6 had IVT-LAF; 4 had anterior fascicular involvement only, while 2 had both anterior and posterior fascicular involvement. All 6 of them underwent RFCA at the median age of 8.8 years (range 4.3-14.3 years). RFCA was successful in all patients, but 4 had recurrence and underwent 1-3 additional sessions of RFCA. In a total of 10 RFCA sessions, the overall recurrence rate was 50%. The site of RFCA was determined on the basis of detection of diastolic potential during ventricular tachycardia (7 sessions) or isolated delayed potential during sinus rhythm (1) or by pace mapping (2). During the median follow-up period of 33 months, no further recurrence was reported except for 1 patient, who had a recurrence and was scheduled for additional session at the time of this report. Major complications included 1 case of complete atrioventricular block and 1 case of complete left bundle branch block. CONCLUSION: Despite a high recurrence rate and a few complications, RFCA of the site of isolated delayed potential or diastolic potential, if applied cautiously, is a possible treatment of choice for pediatric IVT-LAF.

[A case report of distal gastrectomy with thrombectomy after NAC for Gastric cancer].

This is a case report of gastric cancer with a tumor embolus in the portal vein of a 76-year-old male. Both computed tomography (CT) and upper gastrointestinal endoscopy were performed. The diagnosis was gastric cancer with an accompanying tumor embolus in the portal vein, specifically in the superior mesenteric vein. After neoadjuvant chemotherapy, a distal gastrectomy, and thrombectomy were performed. Upon pathological examination, the main tumor was diagnosed as adenocarcinoma, and the embolus was confirmed to extend from the main tumor into the superior mesenteric vein. Upon immunostaining examination, neither the embolus nor main tumor expressed alpha-fetoprotein (AFP), but both expressed carcinoembryonic antigen (CEA). Gastric cancer with a tumor embolus in the portal vein is considered an incurable disease. However, with no other non-curative factor than portal vein embolus, it is possible that gastrectomy with thrombectomy can result in a good prognosis. On the other hand, it is extremely difficult to improve the prognosis of gastric cancer with both tumor embolus in the portal vein and liver metastasis.

[A case of severe hand-foot syndrome caused by capecitabine].

A 55-year-old woman underwent total mastectomy and axillary lymphnode dissection in 2001. Widespread lymphnode metastasis was found histologically (26/33). Neither PgR nor ER was positive. She underwent an AC regimen and paclitaxel chemotherapy. As CEA began to rise in 2002, she was given paclitaxel and docetaxel chemotherapy sequentially. As CEA rose again in 2004, capecitabine was begun. Painful erythema of the palms and soles of the feet appeared at the end of the second cycle. After admission, severe bone marrow suppression and jaundice were found. The bilateral hands, palms and soles of the feet became bullous and erosive with desquamation. The erosive lesions began to heal with epithelization in the third week. After general conditions had improved, capecitabine was restarted at a reduced dose. This patient had continued taking capecitabine even though she noticed the occurrence of the adverse effect. Patients and doctors must share confidential information when performing chemotherapy at the outpatient clinic.