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Use of cardiovascular magnetic resonance in the evaluation of a giant right atrial appendage aneurysm: a case report and review of the literature.

BMC Res Notes. 2017 Dec 04;10(1):681

Authors: Sivakumaran L, Sayegh K, Mehanna E, Sanchez FW, Fields J, Cury R

Abstract
BACKGROUND: Right atrial appendage aneurysms are rare entities that may have significant clinical consequences. When co-existing with atrial fibrillation, patients are at risk of developing pulmonary or paradoxical systemic emboli.
CASE PRESENTATION: An elderly patient presented to medical attention with symptoms of acute diverticulitis. On abdominal computed tomography, a massively enlarged right atrial appendage aneurysm was discovered incidentally. The aneurysm caused marked compression of the right ventricle and contained an area of hypoenhancement concerning for an intraluminal thrombus. Gadolinium-enhanced cardiovascular magnetic resonance was performed and first-pass perfusion images demonstrated that the area of hypoenhancement was in fact poorly mixing blood. The patient was therefore managed medically.
CONCLUSION: Right atrial appendage aneurysms are infrequently encountered cardiac abnormalities. In the literature, surgery has been offered to patients who are young, symptomatic, or have evidence of thrombotic disease, although whether this practice pattern is associated with superior clinical outcomes is unclear. In the present case, gadolinium-enhanced cardiovascular magnetic resonance imaging was used to exclude the presence of intraluminal thrombus in an elderly patient, which helped orient the patient's treating team towards medical-rather than surgical-therapy.

PMID: 29202817 [PubMed - indexed for MEDLINE]

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Near to One's Heart: The Intimate Relationship Between the Placenta and Fetal Heart.

Front Physiol. 2018;9:629

Authors: Camm EJ, Botting KJ, Sferruzzi-Perri AN

Abstract
The development of the fetal heart is exquisitely controlled by a multitude of factors, ranging from humoral to mechanical forces. The gatekeeper regulating many of these factors is the placenta, an external fetal organ. As such, resistance within the placental vascular bed has a direct influence on the fetal circulation and therefore, the developing heart. In addition, the placenta serves as the interface between the mother and fetus, controlling substrate exchange and release of hormones into both circulations. The intricate relationship between the placenta and fetal heart is appreciated in instances of clinical placental pathology. Abnormal umbilical cord insertion is associated with congenital heart defects. Likewise, twin-to-twin transfusion syndrome, where monochorionic twins have unequal sharing of their placenta due to inter-twin vascular anastomoses, can result in cardiac remodeling and dysfunction in both fetuses. Moreover, epidemiological studies have suggested a link between placental phenotypic traits and increased risk of cardiovascular disease in adult life. To date, the mechanistic basis of the relationships between the placenta, fetal heart development and later risk of cardiac dysfunction have not been fully elucidated. However, studies using environmental exposures and gene manipulations in experimental animals are providing insights into the pathways involved. Likewise, surgical instrumentation of the maternal and fetal circulations in large animal species has enabled the manipulation of specific humoral and mechanical factors to investigate their roles in fetal cardiac development. This review will focus on such studies and what is known to date about the link between the placenta and heart development.

PMID: 29997513 [PubMed]

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Clinical and Parental Status of Patients with Congenital Heart Disease Associated Pulmonary Arterial Hypertension.

Isr Med Assoc J. 2017 08;19(8):489-493

Authors: Nir A, Berkman N

Abstract
BACKGROUND: Pulmonary arterial hypertension (PAH) is a significant consequence of congenital heart disease (CHD). Its presence and severity is associated with increased morbidity and mortality.
OBJECTIVES: To evaluate the clinical and demographic characteristics of adults with congenital heart diseases (ADCHD) and PAH at a single center.
METHODS: A prospective registry of all patients with PAH was conducted between 2009 and 2015.
RESULTS: Thirty-two patients were identified. The mean age at the last visit was 44 years (range 19-77 years). The prevalence of PAH among all ADCHD patients was 6% (95% confidence interval 4.3%-8.4%). A much higher prevalence (53%) was found in patients with Down syndrome. Most patients with PAH had moderate or severe disease. Fifteen patients (47%) were treated with pulmonary vasodilators and 6 (19%) with combination therapy. The average World Health Organization functional class was 2.6. Morbidity included cerebral vascular accident or transient ischemic attack in 22% (mostly in patients with right-to-left shunt) and arrhythmia in 37% of the patients. During a median follow-up of 3.5 years, 5 patients (15.6%) died. Of 13 women with no mental retardation, 11 were or had been married and all had children (between 1 and 13, mean 3.3).
CONCLUSIONS: Patients with congenital heart disease and PAH have significant morbidity and mortality. PAH is more prevalent in patients with Down syndrome. While pulmonary pressure during the reproductive years was not always known, 27% of women with PAH at the time of the study were multiparous.

PMID: 28825767 [PubMed - indexed for MEDLINE]

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Early outcomes of percutaneous pulmonary valve implantation using the Edwards SAPIEN XT transcatheter heart valve system.

Int J Cardiol. 2018 Jan 01;250:86-91

Authors: Haas NA, Carere RG, Kretschmar O, Horlick E, Rodés-Cabau J, de Wolf D, Gewillig M, Mullen M, Lehner A, Deutsch C, Bramlage P, Ewert P

Abstract
BACKGROUND: Patients with congenital or acquired heart defects affecting the pulmonary valve and right ventricular outflow tract (RVOT) commonly require multiple surgical interventions, resulting in significant morbidity. A less invasive alternative is percutaneous pulmonary valve implantation (PPVI). Though studies have previously reported the safety and efficacy of the early generation transcatheter heart valves (THVs), data on more recent devices are severely lacking.
METHODS AND RESULTS: We performed a multinational, multicentre, retrospective, observational registry analysis of patients who underwent PPVI using the Edwards SAPIEN XT THV. Of the 46 patients that were enrolled, the majority had tetralogy of Fallot as the underlying diagnosis (58.7%), and stentless xenograft as the most common RVOT anatomy (34.8%). Procedural success rate was high (93.5%), with a low frequency of periprocedural complications and adverse events (6.5% and 10.9%, respectively). At 30days post-procedure, NYHA class had improved significantly (90.6% were at NYHA I or II). The rate of moderate/severe pulmonary regurgitation had decreased from 76.1% at baseline to 5.0% at 30days, and the calculated peak systolic gradient had decreased from 45.2 (SD±21.3) mmHg to 16.4 (SD±8.0) mmHg, with these values remaining low up to 2years.
CONCLUSIONS: The data suggest the efficacy and safety of the SAPIEN XT THV in PPVI in common anatomies in patients with conduits, as well as those with native pulmonary valves or transannular patches. Continued data collection is necessary to verify long-term findings. CLINICALTRIALS.
GOV IDENTIFIER: NCT02302131.

PMID: 29017776 [PubMed - indexed for MEDLINE]

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Quality of life after surgical treatment of coarctation in long-term follow-up (CoAFU): Predictive value of clinical variables.

Int J Cardiol. 2018 Jan 01;250:116-119

Authors: Bambul Heck P, Pabst von Ohain J, Kaemmerer H, Ewert P, Hager A

Abstract
BACKGROUND: We sought to analyze the quality of life and the predictive value of clinical variables from previous follow-up study in patients late after surgical treatment of aortic coarctation on the quality of life.
METHODS: All patients, who have participated in the prospective cross-sectional COALA Study in 2000 with a structural clinical investigation including blood pressure measurement and symptom-limited exercise test were contacted for the health-related quality of life questionnaire SF-36 from January 2013 through December 2014.
RESULTS: From 273 eligible patients, we received data from 135 patients, 9 of them died during the follow-up time at the median age of 46years (range 30-64years). Seventy-four patients did not participate in the study, other 64 patients moved to remote or unknown areas and could not be contacted. Quality of life was good in the fields of physical role and pain. However, patients reported a significant impairment in general health and in health transition, depending on the age. Arterial hypertension and variables from echocardiography or exercise testing from the COALA study were not predictive on functional health status.
CONCLUSION: Quality of life in patients late after aortic coarctation repair is fairly good compared with healthy controls. Impairments in general health and health transition depend mainly on age, can be explained due to numerous comorbidities and reinterventions in long-term. The predictive value of the commonly assessed clinical variables on quality of life is limited.

PMID: 29042092 [PubMed - indexed for MEDLINE]

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Autonomic Reflex Screen Test Abnormalities in Cold-Induced Sweating Syndrome Type 1.

J Clin Neuromuscul Dis. 2017 Dec;19(2):76-79

Authors: El-Dokla AM, Ferdous J, Ali ST, Alam K

Abstract
Cold-induced sweating syndrome (CISS) is a rare autosomal recessive disease due to mutation in the Cytokine receptor-like factor 1 (CRLF1). The characteristic symptom of CISS is the tendency to sweat profusely especially in the upper body and hands when the patient is exposed to cold temperature. We sought to first report the findings of autonomic reflex screen in a case of CISS type 1 with Cytokine receptor-like factor 1 mutation. Valsalva morphology, Valsalva ratio, and heart rate response to deep breathing were normal for the patient's age. Quantitative sudomotor axon reflex test showed nonlength dependent decrease in the sweat volume. Tilt table revealed evidence of reflex (vasovagal) "syncope," however, the patient was asymptomatic without loss of consciousness.

PMID: 29189552 [PubMed - indexed for MEDLINE]

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Massive myocardial infarction due to the complete occlusion of the left anterior descending coronary artery after blunt chest trauma.

Cardiovasc Revasc Med. 2018 Jul 07;:

Authors: Zuin M, Rigatelli G, Fogato L, Faggian G, Zuliani G, Roncon L

PMID: 30006241 [PubMed - as supplied by publisher]

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Short-term cardiopulmonary efficiency improvement after transcatheter baffle leak closure in a Mustard-operated patient.

J Cardiovasc Med (Hagerstown). 2017 06;18(6):447-449

Authors: Mezzani A, Butera G, Chessa M, Micheletti A, Negura D, Calaciura R, Carminati M

PMID: 23314595 [PubMed - indexed for MEDLINE]

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A Rare Cause of Exercise Intolerance and Progressive Dyspnea in an Elderly Woman.

J Cardiothorac Vasc Anesth. 2017 02;31(1):391-394

Authors: Steck DT, Tawil JN, Boettcher BT, Pagel PS

PMID: 27554233 [PubMed - indexed for MEDLINE]

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Percutaneous management of failed bioprosthetic pulmonary valves in patients with congenital heart defects.

J Cardiovasc Med (Hagerstown). 2017 Jun;18(6):430-435

Authors: Tarzia P, Conforti E, Giamberti A, Varrica A, Giugno L, Micheletti A, Negura D, Piazza L, Saracino A, Carminati M, Chessa M

Abstract
AIMS: We reviewed our center experience in the field of transcatheter pulmonary valve-in-valve implantation (TPViV), that is emerging as a treatment option for patients with pulmonary bioprosthetic valve (BPV) dysfunction.
METHODS: Between April 2008 and September 2015, a total of six patients with congenital heart disease (four men) underwent TPViV due to stenosis of preexisting BPV. Four patients received a Melody Medtronic Transcatheter Pulmonary Valve and two an Edward Sapien Valve.
RESULTS: No procedural-related complications occurred. After valve implantation, right ventricular systolic pressure (RVSP, 80.5 ± 25.3-41.2 ± 8.35 mmHg, P < 0.05), right ventricular outflow tract (RVOT) gradient (55.3 ± 23.4-10.6 ± 3.8 mmHg, P < 0.05), and RVSP-to-aortic pressure (0.75 ± 0.21-0.38 ± 0.21, P = 0.01) fell significantly. Echocardiograms at follow-up revealed a significant reduction in estimated RVSP (88.7 ± 22-21.7 ± 4.7 mmHg, P < 0.05), in RVOT (76.2 ± 17.9-25.7 ± 6.1 mmHg, P = 0.005), and in mean RVOT (40.7 ± 9.9-15.5 ± 4.8 mmHg, P < 0.05) gradients. Cardiac magnetic resonance showed no significant change in biventricular dimensions and function. Symptomatic patients reported improvement of symptoms, although cardiopulmonary exercise did not show any significant differences.
CONCLUSION: TPViV is an effective and well tolerated treatment for BPV dysfunction, improving freedom from surgical reintervention. Long-term studies will redefine the management of dysfunctional RVOT, either native or surrogate.

PMID: 27828833 [PubMed - indexed for MEDLINE]

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