Quantitative thallium-201 myocardial imaging in assessing right ventricular pressure in patients with congenital heart defects.

Abstract Background A parachute tricuspid valve is a very rare congenital cardiac anomaly. Its morphological features and clinical implications have not been sufficiently described so far. The purpose of the present systematic review is to disclose the morphological and clinical characteristics of parachute tricuspid valve, and to discuss its diagnostic methods, treatments and patients’ outcomes. Main body The Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement guidelines were followed in this systematic review. Publications were systematically searched in the PubMed, Highwire Press, and the Cochrane Library databases. By comprehensive retrieval of the pertinent literature published between 1979 and 2019, 13 reports were collected with 14 patients recruited into this study. Their ages ranged from neonate to 52 years old with a median age of 23 years. Tricuspid valve regurgitation of a less-than-severe degree was seen in 6 (60%) patients, tricuspid valve stenosis was present in 3 (30%) patients and normally functioning tricuspid valve was noted in 1 (10%) patient. All patients had a single papillary muscle in the right ventricle. The chordae tendineae could be normal in length and thickness, or elongated, or shortened and thickened. Forty percent of the patients were asymptomatic or with only mild symptoms and did not need a surgical or interventional therapy, and 6 (60%) patients were indicated for a surgical/interventional treatment due to their severe presenting symptoms, associated congenital heart defects, and the resultant severe right ventricular inflow obstruction and (or) tricuspid stenosis. Patients’ outcomes varied depending on the substantial status of the patients with a survival rate of 70% and mortality rate of 30%. Conclusion A few patients with a parachute tricuspid valve are asymptomatic or only with mild symptoms and a surgical or interventional treatment is not required. The surgical/interventional indications for parachute tricuspid valve patients are their severe presenting symptoms, associated congenital heart defects, and the resultant severe right ventricular inflow obstruction and (or) tricuspid stenosis. The survival rate of this patient setting is satisfactory.

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Increased plasma levels of natriuretic peptide type B and A in children with congenital heart defects with left compared with right ventricular volume overload or pressure overload

Clinical Physiology and Functional Imaging ◽

10.1111/j.1475-097x.2005.00622.x ◽

2005 ◽

Vol 25 (5) ◽

pp. 263-269 ◽

Cited By ~ 36

Author(s):

Daniel Holmgren ◽

Andreas Westerlind ◽

Per-Arne Lundberg ◽

Hakan Wahlander

Keyword(s):

Right Ventricular ◽

Congenital Heart Defects ◽

Plasma Levels ◽

Congenital Heart ◽

Heart Defects ◽

Pressure Overload ◽

Volume Overload ◽

Ventricular Volume ◽

Right Ventricular Volume ◽

Type B

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Physiological and Clinical Consequences of Relief of Right Ventricular Outflow Tract Obstruction Late After Repair of Congenital Heart Defects

Circulation ◽

10.1161/circulationaha.105.591438 ◽

2006 ◽

Vol 113 (17) ◽

pp. 2037-2044 ◽

Cited By ~ 115

Author(s):

Louise Coats ◽

Sachin Khambadkone ◽

Graham Derrick ◽

Shankar Sridharan ◽

Silvia Schievano ◽

...

Keyword(s):

Right Ventricular ◽

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Defects ◽

Right Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Outflow Tract ◽

Ventricular Outflow Tract Obstruction ◽

Clinical Consequences

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Comparison of Right Ventricular Stroke Work for Tetralogy Patient and Normal Subject

ASME 2008 Summer Bioengineering Conference, Parts A and B ◽

10.1115/sbc2008-193145 ◽

2008 ◽

Author(s):

Ashish Das ◽

William Gottliebson ◽

Rupak K. Banerjee

Keyword(s):

Right Ventricular ◽

Congenital Heart Defects ◽

Congenital Heart ◽

Pulmonary Valve ◽

Heart Defects ◽

Pulmonary Stenosis ◽

The United States ◽

Stroke Work ◽

Loading Conditions ◽

Clinical Issue

Tetralogy of Fallot (TOF), also called blue-baby syndrome is one of the most common congenital heart defects in children after infancy and is estimated to account for 10% of all congenital heart defects [3]. TOF consists of four interrelated lesions: i) ventricular septal defect ii) Pulmonary stenosis iii) Right ventricular (RV) hypertrophy and (iv) Overriding Aorta [3]. TOF has been successfully repaired for several decades (Fig. 1). There are now an estimated 100,000 adult “repaired TOF” patients in the United States alone. As a result, long-term sequelae of the disease and repair have become important clinical issue. Specifically, residual pulmonary valve insufficiency (PI) is one such accepted and often unavoidable sequela. PI, when severe, abnormally alters the RV loading conditions, thereby triggering RV hypertrophy and dilatation. In turn, RV dilatation can evolve into irreversible RV myocardial contractile dysfunction, and has been related to sudden death in many “repaired TOF” patients. To normalize RV loading conditions, pulmonary valve replacement is often necessary and should be performed prior to the onset of irreversible RV myocardial damage.

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