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Isolated right ventricular apical hypoplasia characterized by computed tomography and echocardiography.

J Clin Ultrasound. 2018 Jan;46(1):82-84

Authors: Zhou D, Liao HQ, Ouyang MZ, Shang QL, Zhang M

Isolated right ventricular apical hypoplasia is an unusual congenital heart disease that has been mentioned in only one report to our knowledge. We describe the case of a 62-year-old male patient suffering from recurrent abdominal distention, nausea, and lower extremity edema. The right ventricular morphologic abnormalities as shown by echocardiography and CT were comparable to those of left ventricular apical hypoplasia, suggesting right ventricular apical hypoplasia. However, this speculative diagnosis remains to be confirmed by additional cases. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 46:82-84, 2018.

PMID: 28370066 [PubMed - indexed for MEDLINE]

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Is selective echocardiography in duodenal atresia the future standard of care?

J Pediatr Surg. 2017 Dec;52(12):1952-1955

Authors: Khan A, Tanny ST, Perkins EJ, Hunt RW, Hutson JM, King SK, Jones B, Teague WJ

BACKGROUND: Duodenal atresia (DA) is associated with cardiac defects that may have perioperative care implications. Standard preoperative care includes echocardiography to identify such cardiac defects, but this dogma has been challenged. We aimed to assess selective and selective strategies for preoperative echocardiography in DA patients.
METHODS: Single-center retrospective review of neonates with DA over a 16-year period was performed. Data included preoperative cardiovascular and respiratory examination, chest x-ray, and echocardiography. We compared the current nonselective versus selective strategies, limiting preoperative echocardiogram to those in whom: (1) cardiac or respiratory or chest x-ray examination was abnormal, or (2) cardiac or respiratory examination was abnormal. Sensitivity, specificity, positive and negative predictive values were compared with chi-square tests.
RESULTS: Seventy-one of 109 (65%) consecutive neonates with DA underwent preoperative echocardiography according to a nonselective, physician-determined strategy. Forty of 71 (56%) patients had cardiac defects, including 16/40 (27%) major defects. Sixteen additional postoperative echocardiograms revealed 2 missed major defects. In the same cohort, selective strategies would have performed 17-24% fewer echocardiograms without significant detriment in performance.
CONCLUSIONS: All strategies considered missed some major cardiac defects. A selective strategy, determining DA patients not requiring preoperative echocardiogram, could reduce the number of echocardiograms performed without compromising patient safety.
TYPE OF STUDY: Retrospective study.

PMID: 28919320 [PubMed - indexed for MEDLINE]

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EPAS1 Mutations and Paragangliomas in Cyanotic Congenital Heart Disease.

N Engl J Med. 2018 03 29;378(13):1259-1261

Authors: Vaidya A, Flores SK, Cheng ZM, Nicolas M, Deng Y, Opotowsky AR, Lourenço DM, Barletta JA, Rana HQ, Pereira MA, Toledo RA, Dahia PLM

PMID: 29601261 [PubMed - indexed for MEDLINE]

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Three-vessel-trachea view in the diagnosis of fetal cardiac great vessel malformation.

J Biol Regul Homeost Agents. 2018 Mar-Apr;32(2):351-355

Authors: Chen KB, Gu Q, Xia T, Lu X, Zhang ZD

Fetal cardiac great vessel malformation is attracting increasing attention in the prenatal ultrasonic diagnosis of fetal congenital heart disease. To investigate the clinical diagnostic values of three-vessel-trachea view (3VT view) in the ultrasonic diagnosis of this malformation, the present study analyzed the echocardiographic examination results of 77 fetuses with great vessel malformation, retrospectively analyzed the echocardiographic characteristics in the three-vessel-trachea view, and followed up the enrolled cases. The results suggest that great vessel malformation had characteristic manifestations, such as abnormal arrangement order, inner diameter, blood flow direction and branch. Color Doppler flow imaging found V, O, C, U, Ioo and oVo structures. There were 20 cases of blood vessel position abnormality, 38 cases of abnormal blood vessel diameter, and 19 cases of abnormal number of blood vessels. The detection rate of abnormal blood vessel diameter was 95%, which was the highest; the detection rate of abnormal blood vessel position was 97.4%, and that of abnormal number of blood vessels was 84.2%. It is concluded that the 3VT view can indicate fetal cardiac great vessel malformation. The 3VT view is beneficial to timely prenatal diagnosis, relief of body pain and improvement of quality of birth.

PMID: 29685018 [PubMed - indexed for MEDLINE]

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Gestational diabetes influences the expression of hypertrophic genes in left ventricle of rat's offspring.

Iran J Basic Med Sci. 2018 May;21(5):525-528

Authors: Kermani ES, Nazari Z, Mehdizadeh M, Shahbazi M, Golalipour MJ

Objectives: Gestational diabetes increases the risk of congenital heart disease in the offspring, but the molecular mechanism underlying this process remains unclear. Therefore, the current study was conducted to assess the effects of induced gestational diabetes on expression of some involved genes in cardiac hypertrophy in the offspring of diabetic rats.
Materials and Methods: Diabetes was induced in 40 adult Wistar rats by intraperitoneal injection of 45 mg/kg of streptozotocin. The day of appearance of the vaginal plug was assumed as day zero of gestation for inducing diabetes. After pregnancy, the offspring was maintained until they reach the age of 12 weeks. Then, their hearts were excised and were sectioned for molecular study. We analyzed the expression pattern of some hypertrophic genes by the quantitative real-time RT-PCR.
Results: The mRNA expression levels of all studied genes including c-jun, c-fos, c-myc, alpha-myosin heavy chain (α-MHC), atrial natriuretic factor (ANF) and β-MHC, which are important in cardiomyocyte hypertrophy, were higher in the offspring of the diabetic group compared to controls. Significant differences were found for β-MHC and c-myc with P<0.01 and for α-MHC and c-fos with P<0.05.
Conclusion: Gestational diabetes upregulates expression of c-jun, c-fos c-myc, α-MHC, ANF and β-MHC genes that are involved in cardiac hypertrophy in the offspring of diabetic rats.

PMID: 29922434 [PubMed]

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Successful atrial fibrillation ablation without pulmonary vein isolation utilizing focal impulse and rotor mapping in an atriopulmonary Fontan.

HeartRhythm Case Rep. 2018 Jun;4(6):241-246

Authors: Kollengode M, Mathew J, Yeung E, Sauer WH, Nguyen DT

PMID: 29922583 [PubMed]

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Extracranial-intracranial bypass in medial sphenoid ridge meningioma associated with severe stenosis of the intracranial segments of the internal carotid artery: A case report.

Medicine (Baltimore). 2018 Jun;97(24):e11123

Authors: Huang Y, Wang Z, Han Q

RATIONALE: Tumor resection and extracranial-intracranial bypass concerning medial sphenoid ridge meningioma associated with severe stenosis of the internal carotid artery (ICA) of intracranial segments has been rarely presented. Effective treatment as to the complex lesions may be complicated. Tumor resection and cerebrovascular protection should be both taken into consideration.
PATIENT CONCERNS: We presented one case of medial sphenoid ridge meningioma associated with severe stenosis of the internal carotid artery of intracranial segments. The patient suffered hyperthyroidism, mirror-image dextrocardia and congenital heart disease atrial septal defect simultaneously.
DIAGNOSES: Before the neurosurgical treatment , the colleagues of department of cardiac surgery, anesthesiology and respiratory medicine agreed on our plan of resecting the tumor following the comprehensive evaluation of basal clinical conditions in the patient. For reducing the bleeding intraoperatively, the interventional branch performed digital subtraction angiography(DSA) and found collateral anastomosis between the supplying vessels of left middle meningeal arteries and anterior choroid arteries. No preoperative interventional embolization was determined considering the risk of cerebral ischemia.
INTERVENTIONS: The following subtotal resection of medial sphenoid ridge meningioma and left extracranial-intracranial bypass were carried out. Additionally, ipsilateral decompressive craniectomy was done. Post-operative imaging Computed tomography (CT), Computed tomography angiography (CTA) and Transcranial Doppler (TCD) indicated subtotal resection of tumor and bypass patency.
OUTCOMES: The patient was discharged with the right limbs of muscle strength of grade IV. The muscle strength of the patient returned to grade V after 6 months of follow-up.
LESSONS: Comprehensive treatment of tumor resection and extracranial-intracranial bypass concerning medial sphenoid ridge meningioma associated with severe stenosis of the internal carotid artery of intracranial segments is effective.

PMID: 29901637 [PubMed - indexed for MEDLINE]

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Pseudo-Reentry of Atrial Tachycardia After Surgical Repair of Congenital Heart Disease.

J Cardiovasc Electrophysiol. 2016 07;27(7):876-7

Authors: Yokota J, Fujiu K, Kojima T, Komuro I

PMID: 26776918 [PubMed - indexed for MEDLINE]

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Slow Conducting Electroanatomic Isthmuses: An Important Link Between QRS Duration and Ventricular Tachycardia in Tetralogy of Fallot.

JACC Clin Electrophysiol. 2018 Jun;4(6):781-793

Authors: Kapel GFL, Brouwer C, Jalal Z, Sacher F, Venlet J, Schalij MJ, Thambo JB, Jongbloed MRM, Blom NA, de Riva M, Zeppenfeld K

OBJECTIVES: This study sought to evaluate the influence of slow conducting anatomic isthmuses (SCAI) as dominant ventricular tachycardia (VT) substrate on QRS duration.
BACKGROUND: QRS prolongation has been associated with VT in repaired tetralogy of Fallot.
METHODS: Seventy-eight repaired tetralogy of Fallot patients (age 37 ± 15 years, 52 male, QRS duration 153 ± 29 ms, 67 right bundle branch blocks [RBBB]) underwent programmed stimulation and electroanatomic activation mapping during sinus rhythm. Right ventricular (RV) surface, RV activation pattern, RV activation time, conduction velocity at AI, and remote RV sites were determined.
RESULTS: Twenty-four patients were inducible for VT (VT+); SCAI was present in 22 of 24 VT+ but only in 2 of 54 patients without inducible VT (VT-). Conduction velocity through AI was slower in VT+ patients (median of 0.3 [0.3 to 0.4] vs. 0.7 [0.6 to 0.9] m/s; p < 0.01) but conduction velocity in the remote RV did not differ between groups. In non-RBBB, QRS duration was similar in VT+ patients (n = 6) and VT- patients (n = 5), but RV activation within SCAI exceeded QRS offset in VT+ patients (37 ± 20 ms vs. -5 ± 9 ms, p < 0.01). In RBBB, both QRS duration and RV activation time were longer in VT+ patients (n = 18, 17 of 18 QRS > 150 ms) compared with VT- patients (n = 49, 27 of 49 QRS > 150 ms) (173 ± 22 ms vs. 156 ± 20 ms; p < 0.01; 141 ± 22 ms vs. 129 ± 21 ms; p = 0.04). In VT+ patients, QRS prolongation >150 ms (n = 17) was due to SCAI or blocked isthmus in 15 patients (88%) and 1 (6%). In contrast, in VT- patients, QRS prolongation >150 ms (n = 27) was due to enlarged RV or blocked isthmus in 10 patients (37%) and 8 (30%), but due to SCAI in only 1 (4%). After exclusion of a severely enlarged RV, a QRS duration >150 ms was highly predictive for SCAI/blocked AI (OR: 17; 95% CI: 3.3 to 84; p < 0.01).
CONCLUSIONS: A narrow QRS interval does not exclude VT-related SCAI. In the presence of RBBB, SCAI further prolongs QRS duration. QRS duration >150 ms is highly suspicious for SCAI or isthmus block distinguishable by electroanatomic mapping.

PMID: 29929672 [PubMed - in process]

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Potential benefit of a simultaneous, side-by-side display of contrast MDCT and echocardiography over routine sequential imaging for assessment of adult congenital heart disease: A preliminary study.

J Cardiol. 2018 Jun 18;:

Authors: Oe H, Watanabe N, Miyoshi T, Osawa K, Akagi T, Kanazawa S, Ito H

BACKGROUND: Management of adult congenital heart disease (ACHD) patients requires understanding of its complex morphology and functional features. An innovative imaging technique has been developed to display a virtual multi-planar reconstruction obtained from contrast-enhanced multidetector-computed tomography (MDCT) corresponding to the same cross-sectional image from transthoracic echocardiography (TTE). The aim of this study is to assess the usefulness of this imaging technology in ACHD patients.
METHODS: This study consisted of 46 consecutive patients (30 women; mean age, 52±18 years old) with ACHD who had undergone contrast MDCT. All patients underwent TTE within a week of MDCT. An experienced sonographer who did not know the results of MDCT conducted a diagnosis using TTE and, then, using the new imaging technology. We studied whether this imaging technology provided additional or unexpected findings or makes more accurate diagnosis.
RESULTS: In this imaging technology, MDCT cross-section provides higher-resolution image to the deep compared to corresponding TTE image. Depending on the MDCT section which can be arbitrarily set under the echo guide, we can diagnose unexpected or incremental lesions or more accurately assess the severity of the lesion in 27 patients (59%) compared to TTE study alone. This imaging technology was useful in the following situations: CONCLUSIONS: This integrated imaging technology provides incremental role over TTE in complex anatomy, and allows functional information in ACHD patients.

PMID: 29929778 [PubMed - as supplied by publisher]