Early insertion of a valved conduit for chronic pulmonary insufficiency based on right ventricular volumetry helps restoration of normal right ventricular dimensions

Background: Congenital cardiac diseases are a common cause of death in puppies. Tricuspid valve dysplasia is characterized by thickening and displacement of the leaflets of the tricuspid valve, agenesis of the valves, and incomplete separation of valve components. Papillary muscles may fuse and display shortened or absent chordae tendineae that contribute to tricuspid regurgitation. Diagnostic features of tricuspid valve dysplasia include cardiomegaly with massive right atrium enlargement on thoracic radiography and tricuspid insufficiency on an ultrasound. We aimed to describe clinicopathological findings in a dog (Canis familiaris) with tricuspid dysplasia.Case: We aimed to describe tricuspid valve dysplasia in a dog referred for necropsy at the Anatomical Pathology Sector of The Rural Federal University of Rio de Janeiro, Brazil, with a clinical history of abdominal swelling, dyspnea, cyanosis, ascites, and prostration. Echocardiography and abdominal ultrasound revealed right ventricular enlargement, hepatomegaly, and splenomegaly. Examination of the heart showed prominent enlargement, thickening and dilation of the right chambers, thickening of the tricuspid leaflets, and moderately shortened chordae tendineae. The liver was enlarged, with a nutmeg pattern, and foci of clotting and fibrin adhesions in the lateral right lobule.Discussion: Epidemiological, clinical, and pathological findings were consistent with tricuspid valve dysplasia. Although structural abnormalities of the tricuspid and mitral valves are well known in fetuses and neonates, congenital and secondary tricuspid malformations are rare in dogs. The survival rate is associated with the severity of heart lesions. Tricuspid valve dysplasia is mostly observed in large-breed dogs (>20 kg), particularly in Labrador Retrievers, Boxers, and German Shepherds. Regardless, most dogs with tricuspid valve dysplasia are of a pure-breed, which differs from our findings because our dog was a mongrel. Our dog displayed signs of dyspnea, cyanosis, abdominal swelling, prostration, and enlarged liver and spleen on ultrasound examination. Tricuspid valve dysplasia led to heart enlargement and right congestive heart failure, with consequent ascites, abdominal swelling, weakness, lethargy, jugular venous distension, and hepatomegaly. Overall, the heart showed prominent enlargement, thickening and dilation of the right chambers, thickening of the tricuspid leaflets, and moderately shortened chordae tendineae. The liver had a nutmeg pattern. Tricuspid valve dysplasia is characterized by malformation of the tricuspid valve leaflets, chord tendineae, or papillary muscles. Malformed tricuspid valves are known to result in variable degrees of regurgitation, leading to right atrial overflow and ventricular eccentric hypertrophy. Differential diagnosis includes myocarditis, tricuspid valve endocarditis, tricuspid endocardiosis, tricuspid valve prolapse and right ventricular dysplasia, right ventricular enlargement with tricuspidal regurgitation due to pulmonary insufficiency, and arrhythmogenic right ventricular cardiomyopathy. Signs of heart murmurs (irregular sounds of the heart) on clinical examination may indicate an irregular blood flow pattern, and imaging tests may be necessary for assessing the presence and severity of any lesions. The epidemiologic, clinical, and pathological findings were consistent with those of tricuspid valve dysplasia.

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Tetralogy of Fallot

Circulation ◽

10.1161/circ.102.suppl_3.iii-116 ◽

2000 ◽

Vol 102 (suppl_3) ◽

Author(s):

Y. d’Udekem ◽

C. Ovaert ◽

F. Grandjean ◽

V. Gerin ◽

M. Cailteux ◽

...

Keyword(s):

Right Ventricular ◽

Tetralogy Of Fallot ◽

Left Ventricular ◽

Ventricular Dilatation ◽

Pulmonary Insufficiency ◽

Direct Closure ◽

Long Term Outcome ◽

Cox Regression Analysis ◽

Right Ventricular Dilatation

Background —In tetralogy of Fallot, transannular patching is suspected to be responsible for late right ventricular dilatation. Methods and Results —In our institution, 191 patients survived a tetralogy of Fallot repair between 1964 and 1984. Transannular patching was used in 99 patients (52%), patch closure of a right ventriculotomy in 35, and direct closure of a right ventriculotomy in 55. Two had a transatrial-transpulmonary approach. To identify predictive factors of adverse long-term outcome related to right ventricular dilatation, the following events were investigated: cardiac death, reoperation for symptomatic right ventricular dilatation, and NYHA class II or III by Cox regression analysis. Mean follow-up reached 22±5 years. The 30-year survival was 86±5%. Right ventricular patching, whether transannular or not, was the most significant independent predictor of late adverse event (improvement χ 2 =16.6, P <0.001). In patients who had direct closure, the ratio between end-diastolic right and left ventricular dimensions on echocardiography was smaller (0.61±0.017 versus 0.75±0.23, P =0.007), with a smaller proportion presenting severe pulmonary insufficiency (9% versus 40%, P =0.005). There was no difference between right ventricular and transannular patching concerning late outcome (log rank P value=0.6), right ventricular size (0.70±0.28 versus 0.76±0.26, P =0.4), or incidence of severe pulmonary insufficiency (30% versus 43%, P =0.3). Conclusions —In tetralogy of Fallot, transannular patching does not result in a worse late functional outcome than patching of an incision limited to the right ventricle. Both are responsible for a similar degree of long-term pulmonary insufficiency and right ventricular dilatation.

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Improved right ventricular function following late pulmonary valve replacement for residual pulmonary insufficiency or stenosis

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/s0022-5223(19)38661-1 ◽

1985 ◽

Vol 90 (1) ◽

pp. 50-55 ◽

Cited By ~ 122

Author(s):

Edward L. Bove ◽

Rae-Ellen W. Kavey ◽

Craig J. Byrum ◽

Henry M. Sondheimer ◽

Marie S. Blackman ◽

...

Keyword(s):

Right Ventricular ◽

Ventricular Function ◽

Valve Replacement ◽

Right Ventricular Function ◽

Pulmonary Valve ◽

Pulmonary Valve Replacement ◽

Pulmonary Insufficiency

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Postsurgery of Tetralogy of Fallot, Aneurysm of the Right Ventricular Outflow Tract, and Branch Pulmonary Insufficiency

Echocardiographic Atlas of Adult Congenital Heart Disease ◽

10.1007/978-3-319-12934-1_76 ◽

2015 ◽

pp. 245-248

Author(s):

Hakimeh Sadeghian ◽

Zahra Savand-Roomi

Keyword(s):

Right Ventricular ◽

Tetralogy Of Fallot ◽

Right Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Outflow Tract ◽

Pulmonary Insufficiency ◽

The Right

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Long-term results of large-calibre expanded polytetrafluoroethylene-valved conduits with bulging sinuses

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezaa240 ◽

2020 ◽

Vol 58 (6) ◽

pp. 1274-1280

Author(s):

Shuhei Fujita ◽

Masaaki Yamagishi ◽

Takako Miyazaki ◽

Yoshinobu Maeda ◽

Keiichi Itatani ◽

...

Keyword(s):

Right Ventricular ◽

Right Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Outflow Tract ◽

The Body ◽

Long Term Results ◽

Pulmonary Insufficiency ◽

Long Term Outcomes ◽

Expanded Polytetrafluoroethylene

Abstract OBJECTIVES In Japan, homograft and bovine jugular vein are available in very limited institutions for the reconstruction of the right ventricular outflow tract, and handmade expanded polytetrafluoroethylene (ePTFE)-valved conduits have been widely used instead. This study aimed to clarify the long-term outcomes and the durability of the ePTFE-valved conduits purely by narrowing down to those with large sizes to eliminate the influence of the body growth. METHODS Between January 2002 and December 2015, patients who underwent right ventricular outflow tract reconstruction in 34 Japanese institutions using ePTFE-valved conduits with a diameter of ≥18 mm were included. All the valved conduits were made in the authors’ institution and delivered to each participating institution. RESULTS Overall, 502 patients were included. Early mortality was 1.4% and not related to conduit failure. The overall survival rate was 98.2% at 5 years and 96.6% at 10 years. Freedom from conduit explantation was 99.5% at 5 years and 89.0% at 10 years. Three patients (0.13 per 100 patient-years) developed infective endocarditis of the conduit, and only 1 patient required conduit removal. Pulmonary insufficiency was mild or less in 480 (96%) patients, and conduit stenosis was mild or less in 436 (88%) patients at the latest follow-up. CONCLUSIONS By narrowing the analyses down to only ePTFE conduits with a large size, satisfactory long-term outcomes of these conduits with a fan-shaped valve and bulging sinuses were shown. These conduits would be among the optimal choices for right ventricular outflow tract reconstruction.

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