scholarly journals Holt-Oram Syndrome in Adult Presenting with Heart Failure: A Rare Presentation

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Rupesh Kumar ◽  
Subhendu Sekhar Mahapatra ◽  
Monalisa Datta ◽  
Amanul Hoque ◽  
Swarnendu Datta ◽  
...  
Keyword(s):  
Heart Failure ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Sporadic Case ◽  
Cardiac Abnormality ◽  
Ventricular Septal Defects ◽  
Rare Presentation ◽  
Live Births ◽  
Septal Defects ◽  
Limb Malformations

Holt-Oram syndrome is a rare inherited disorder involving the hands, arms, and the heart. The defects involve carpal bones of the wrist and the thumb and the associated cardiac anomalies like atrial or ventricular septal defects. Congenital cardiac and upper-limb malformations frequently occur together and are classified as heart-hand syndromes. The most common amongst the heart-hand disorders is the Holt-Oram syndrome, which is characterized by septal defects of the heart and preaxial radial ray abnormalities. Its incidence is one in 100,000 live births. Approximately three out of four patients have some cardiac abnormality with common associations being either an atrial septal defect or ventricular septal defect. Herein, we report a rare sporadic case of Holt-Oram syndrome with atrial septal defect with symptoms of heart failure in a forty-five-year-old lady who underwent emergency cardiac surgery for the symptoms.

Fulminant cytomegalovirus myocarditis in an infant with concomitant large atrial and ventricular septal defects: medical intervention strategy for functional cardiac regeneration

2018 ◽  
Vol 29 (3) ◽  
pp. 277-279
Author(s):  
Roland Schrewe ◽  
Anoosh Esmaeili ◽  
Kachina Behnke-Hall ◽  
Dietmar Schranz
Keyword(s):  
Heart Failure ◽  
Ventricular Septal Defect ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Young Infant ◽  
Intervention Strategy ◽  
Disease Process ◽  
Medical Intervention ◽  
Ventricular Septal Defects ◽  
Septal Defects

AbstractA cytomegalovirus-associated heart failure in a young infant with atrial and ventricular septal defects is reported in this case report. The patient recovered by an anti-congestive and anti-viral therapy with an extra percutaneous transcatheter treatment strategy. In the context of bi-ventricular predominant right heart failure associated with supra-systemic pulmonary hypertension, the already closed arterial duct was re-opened and stented to unload the right ventricle and thereby augment the systemic blood flow. Either the left-to-right shunting atrial septal defect or bi-directional shunting ventricular septal defect was involved in the disease process and was not able to avoid global heart failure. After clinical improvement, the stented duct was shunted left-to-right and was occluded with an ADO-II-AS. During the same procedure the atrial septal defect was closed with an Amplatzer-ASD occluder, while the peri-membranous ventricular septal defect was closed with an ADO-II occluder 2 months later.

Increased Prevalence of Ventricular Septal Defect: Epidemic or Improved Diagnosis

PEDIATRICS ◽  
1989 ◽  
Vol 83 (2) ◽  
pp. 200-203
Author(s):  
Gerard R. Martin ◽  
Lowell W. Perry ◽  
Charlotte Ferencz
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Ductus Arteriosus ◽  
Coarctation Of The Aorta ◽  
Cardiovascular Malformations ◽  
Ventricular Septal Defects ◽  
Live Births ◽  
Septal Defects ◽  
Clinical Diagnoses ◽  
Control Study

The Baltimore-Washington Infant Study is an ongoing case-control study of congenital cardiovascular malformations in infants in whom the clinical diagnoses have been confirmed by echocardiography, catheterization, surgery, or autopsy. An increase in the prevalence of ventricular septal defects was detected in 1,494 infants with congenital cardiovascular malformations between 1981 and 1984. The prevalence of congenital cardiovascular malformations increased from 3.6 to 4.5 per 1,000 live births (P<.025) and the prevalence of ventricular septal defect increased from 1.0 to 1.6 per 1,000 live births (P< .001). The increase in ventricular septal defects accounted for the total increase in congenital cardiovascular malformations. The prevalence of isolated ventricular septal defect increased from 0.67 to 1.17 per 1,000 live births (P<.001). The prevalence of ventricular septal defect with associated coarctation of the aorta, patent ductus arteriosus, atrial septal defect, and pulmonic stenosis did not change. The prevalence of ventricular septal defect diagnosed by catheterization, surgery, and autopsy did not change; however, defects diagnosed by echocardiography increased from 0.30 to 0.70 per 1,000 live births (P<.001). It is concluded that the reported increase in prevalence of ventricular septal defect is due to improved detection of small, isolated ventricular septal defects and that there is no evidence of an "epidemic."

scholarly journals Catheter-based closure of a large atrial septal defect with inferior rim deficiency using pulmonary vein slide-out assisted implantation technique: a case report

2021 ◽  
Vol 5 (2) ◽  
Author(s):  
Salvatore Evola ◽  
Oreste Fabio Triolo ◽  
Giuseppina Novo ◽  
Eustaquio Maria Onorato
Keyword(s):  
Heart Failure ◽  
Atrial Septal Defect ◽  
Pulmonary Vein ◽  
Septal Defect ◽  
Transcatheter Closure ◽  
Implantation Technique ◽  
Secundum Atrial Septal Defect ◽  
Septal Defects ◽  
Case Summary ◽  
Implantation Techniques

Abstract Background Transcatheter approach for large and complex atrial septal defects may represent a therapeutic challenge, particularly when the postero-inferior rim is deficient and floppy. Case summary Here, we describe a successful catheter-based closure of a large (>30 mm) secundum atrial septal defect associated with postero-inferior rim deficiency in a 35-year-old female with congestive heart failure using pulmonary vein slide-out assisted implantation technique. Discussion Inferior–posterior rim deficiency is a well-known risk factor for device instability or embolization. Transcatheter closure may represent a safe and effective alternative to the traditional surgical approach provided that modified implantation techniques are employed.

Levoatriocardinal Vein With Multiple Ventricular Septal Defects and Without Left-Sided Valvular Atresia

2021 ◽  
Vol 12 (1) ◽  
pp. 128-130
Author(s):  
Kartik Patel ◽  
Deepti Kakkar ◽  
Chandrasekaran Ananthnarayan ◽  
Ravi Patel ◽  
Dinesh Patel ◽  
...  
Keyword(s):  
Mitral Regurgitation ◽  
Ventricular Septal Defect ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Ventricular Septal Defects ◽  
Muscular Ventricular Septal Defect ◽  
Septal Defects

Levoatriocardinal vein without left-sided valvular atresia is rare. We hereby present an image of the levoatriocardinal vein in a patient with multiple muscular ventricular septal defect with small atrial septal defect and mitral regurgitation.

scholarly journals Spontaneous Resolution of Residual Shunting in 2 Compromised Patients after Amplatzer Occlusion of Postinfarction Ventricular Septal Defects

2019 ◽  
Vol 46 (1) ◽  
pp. 44-47
Author(s):  
Takashi Yanagiuchi ◽  
Norio Tada ◽  
Taro Suchi ◽  
Yukiko Mizutani ◽  
Takashi Matsumoto ◽  
...  
Keyword(s):  
Heart Failure ◽  
Septal Defect ◽  
Transcatheter Closure ◽  
Severe Heart Failure ◽  
Effective Management ◽  
Ventricular Septal Defects ◽  
Surgical Closure ◽  
Septal Defects ◽  
Gradual Disappearance ◽  
Intensively Managed

Ventricular septal defect (VSD) is a rare, potentially fatal complication of acute myocardial infarction. When surgical closure is contraindicated, transcatheter closure may be an alternative. Residual shunting after transcatheter closure of postinfarction VSDs has been reported; however, we found few cases of this in patients who also had severe heart failure or hemolysis. We report 2 closures of postinfarction VSDs with use of the Amplatzer Septal Occluder. Both elderly patients—one with severe heart failure, one with persistent hemolysis, and neither a surgical candidate—had high-velocity residual shunting through the occluders. We intensively managed the patients' conditions and used angiography and transthoracic echocardiography to record the gradual disappearance of each shunt over 4 months—the first such serial monitoring of which we are aware. We think that even substantial shunting in the presence of severe heart failure or hemolysis can eventually resolve spontaneously, assuming effective management of the concomitant medical conditions.

Epidemiology and diagnosis of ventricular septal defect in Malta

1998 ◽  
Vol 8 (3) ◽  
pp. 329-336 ◽  
Author(s):  
Victor Grech
Keyword(s):  
Ventricular Septal Defect ◽  
Congenital Heart ◽  
Septal Defect ◽  
Significant Decline ◽  
Small Island ◽  
Ventricular Septal Defects ◽  
Live Births ◽  
True Prevalence ◽  
Septal Defects ◽  
Congenital Heart Malformations

AbstractMalta is a small island with minimal changes in its population, making it an ideal location for epidemiological and historical studies dealing with congenital heart malformations. Ventricular septal defect was studied retrospectively from 1930 to 1994. A sharp and significant decline in age at diagnosis was found, predating echocardiography. All defects are now diagnosed by echocardiography, which has resulted in an increased prevalence of this lesion as seen at birth, particularly of minor defects. The prevalence of ventricular septal defect from 1990 to 1994 was 3.85/1000 live births, with 3.O3/1OOO not needing surgery and 0.83/1000 requiring operative intervention. Half the defects closed spontaneously. The majority of ventricular septal defects overall, and those spontaneously closing, were muscular defects. The overall prevalence at birth was significantly higher than that reported in recent studies using similar methodologies, implying that the reported rate is more likely to be the true prevalence at birth of clinically detectable defects.

Does N-terminal pro-brain natriuretic peptide correlate with measured shunt fraction in children with septal defects?

2015 ◽  
Vol 26 (3) ◽  
pp. 469-476
Author(s):  
Abdullah Ozyurt ◽  
Ali Baykan ◽  
Mustafa Argun ◽  
Ozge Pamukcu ◽  
Kazim Uzum ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Brain Natriuretic Peptide ◽  
Atrial Septal Defect ◽  
Natriuretic Peptide ◽  
Significant Positive Correlation ◽  
Septal Defect ◽  
Ventricular Septal Defects ◽  
Positive Correlation ◽  
Haemodynamic Parameters ◽  
Septal Defects

AbstractBackgroundThe aim of this study was to investigate the potential role of N-terminal pro-brain natriuretic peptide in the assessment of shunt severity and invasive haemodynamic parameters in children with atrial septal defects and ventricular septal defects.MethodsThis is a prospective, controlled (n:62), observational study. Correlation analysis was performed between N-terminal pro-brain natriuretic peptide levels and various invasive haemodynamic measurements in 127 children (ventricular septal defect: 64; atrial septal defect: 63). A ratio of pulmonary to systemic blood flow (Qp/Qs⩾1.5) was considered to indicate a significant shunt.ResultsStatistically significant relationship was found between the mean N-terminal pro-brain natriuretic peptide values of the patients, with Qp/Qs⩾1.5 in both defect types and control group. For ventricular septal defect, N-terminal pro-brain natriuretic peptide level⩾113.5 pg/ml was associated with high specificity and sensitivity for determining the significant shunt. In addition, the cut-off point for determining the significant shunt for atrial septal defect was 57.9 pg/ml. Significant positive correlation was found between all invasive haemodynamic parameters and N-terminal pro-brain natriuretic peptide levels in patients with ventricular septal defects. Whereas significant positive correlation was found only between mean pulmonary artery pressure, right ventricular end-diastolic pressure, and systemic pressure to pulmonary pressure ratio and N-terminal pro-brain natriuretic peptide levels in patients with atrial septal defects.ConclusionOur study demonstrated that the N-terminal pro-brain natriuretic peptide measurements could be used as a supporting parameter in determining significance of the shunt.

Delayed Closure of Multiple Muscular Ventricular Septal Defects in an Infant after Coarctation Repair and a Hybrid Procedure�A Case Report

2011 ◽  
Vol 14 (1) ◽  
pp. 67 ◽  
Author(s):  
Ireneusz Haponiuk ◽  
Maciej Chojnicki ◽  
Radosaw Jaworski ◽  
Jacek Juciski ◽  
Mariusz Steffek ◽  
...  
Keyword(s):  
Septal Defect ◽  
Color Doppler ◽  
Hybrid Approach ◽  
Ventricular Septal Defects ◽  
Color Doppler Echocardiography ◽  
Septal Defects ◽  
Coarctation Repair ◽  
Step Procedure ◽  
The Right ◽  
Delayed Closure

There are several strategies of surgical approach for the repair of multiple muscular ventricular septal defects (mVSDs), but none leads to a fully predictable, satisfactory therapeutic outcome in infants. We followed a concept of treating multiple mVSDs consisting of a hybrid approach based on intraoperative perventricular implantation of occluding devices. In this report, we describe a 2-step procedure consisting of a final hybrid approach for multiple mVSDs in the infant following initial coarctation repair with pulmonary artery banding in the newborn. At 7 months, sternotomy and debanding were performed, the right ventricle was punctured under transesophageal echocardiographic guidance, and the 8-mm device was implanted into the septal defect. Color Doppler echocardiography results showed complete closure of all VSDs by 11 months after surgery, probably via a mechanism of a localized inflammatory response reaction, ventricular septum growth, and implant endothelization.

Axillary artery: An unanticipated choice of access in the cath lab!

2021 ◽  
pp. 021849232110264
Author(s):  
Puneet Varma ◽  
Bharath A Paraswanath ◽  
Anand Subramanian ◽  
Jayaranganath Mahimarangaiah
Keyword(s):  
Hospital Stay ◽  
Heart Surgery ◽  
Septal Defect ◽  
Axillary Artery ◽  
Open Heart Surgery ◽  
Open Heart ◽  
Ventricular Septal Defects ◽  
Septal Defects ◽  
Rare Site

Ventricular septal defects are increasingly being closed by transcatheter technique, with lesser morbidity and shorter hospital stay compared to open heart surgery. We report a case of embolization of a duct occluder deployed in a posterior muscular septal defect. The rare site of embolization necessitated an unusual approach for retrieval prior to subsequent closure using a double-disc device.

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