scholarly journals Primary pulmonary valve sarcoma involving pulmonary artery and right ventricular outflow tract

Cor et Vasa ◽  
2015 ◽  
Vol 57 (5) ◽  
pp. e371-e376 ◽  
Author(s):  
Tomáš Toporcer ◽  
Marián Martinček ◽  
Lucia Mistríková ◽  
František Sabol
2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Abdelrahmen Abdelbar ◽  
Raed Azzam ◽  
Kok Hooi Yap ◽  
Ahmed Abousteit

We present a case of a fifty-three-year-old male who presented with severe sepsis. He had been treated as a pneumonia patient for five months before the admission. Investigations revealed isolated pulmonary valve endocarditis and septic pulmonary embolism in addition to undiagnosed right ventricular outflow tract (RVOT) obstruction. The patient underwent surgery for the relief of RVOT obstruction by substantial muscle resection of the RVOT, pulmonary artery embolectomy, pulmonary valve replacement, and reconstruction of RVOT and main pulmonary artery with two separate bovine pericardial patches. He was discharged from our hospital after 6 weeks of intravenous antibiotics. He recovered well on follow-up 16 weeks after discharge. A high-suspicion index is needed to diagnose right-side heart endocarditis. Blood cultures and transesophageal echocardiogram are the key diagnostic tools.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
L L Buttigieg ◽  
K Yamagata ◽  
J Cassar ◽  
M Pace Bardon ◽  
M Balzan ◽  
...  

Abstract Introduction We present an unusual case of sequential right ventricular outflow tract obstruction (RVOTO) caused by pulmonary and subpulmonary stenosis, with formation of aortopulmonary collaterals. Case presentation We report a case of a 47 year old Senegalese man who presented with a three year history of shortness of breath on exertion which worsened over the past week. Cardiovascular examination revealed a parasternal heave, a systolic murmur loudest in the pulmonary area and no evidence of fluid overload. A transthoracic echocardiogram revealed leftward septal deviation during systole due to right ventricular pressure overload, severe right ventricular hypertrophy (RV free wall end-diastolic thickness of 8mm) and normal systolic function (FAC 47%), a dilated right atrium and moderate tricuspid regurgitation with estimated maximum pressures of 112mmHg. On continuous-wave doppler of the right ventricular outflow tract (RVOT), there was a late peaking systolic flow with maximum velocity of 3.8m/s. A cardiac computed tomography (CT) and cardiac magnetic resonance imaging (MRI) was performed which showed sequential stenoses of the RVOT; one at subvalvular level by an infundibular muscular ridge with an area of 0.7 cm2 in mid-systole and one at the level of a dome-shaped pulmonary valve with planimetered valve area of 0.5cm2, severe RV hypertrophy and normal RV ejection fraction. Aortopulmonary collaterals from proximal thoracic descending aorta were seen. The main pulmonary artery was shown to be dilated with no evidence of pulmonary artery branch stenosis. See image: In-plane phase contrast velocity flow mapping of the RVOT using a VENC of 80cm/s, showing aliasing at the level of the pulmonary valve (red arrow) and at the level of the infundibulum (white arrow). Conclusion Congenital pulmonary stenosis (PS) occurs in 8% of congenital heart defects. 80% of pulmonary valve stenosis is typically dome-shaped with commissural fusion whilst the remainder is of the dysplastic type. Congenital PS can be associated with RVOTO at the infundibular level secondary to reactive muscular hypertrophy. In our case, there is a discrete, circumferential muscular ridge at the level of the infundibulum resulting in sequential outflow tract obstruction. PS with intact ventricular septum occurs in conjunction with varying degrees of right ventricular hypertrophy and elevated right ventricular systolic pressures. A dilated pulmonary artery is common in dome-shaped subtype of PS. Survival into adulthood of severe PS is primarily dependent on the adequacy of pulmonary blood flow from systemic-to-pulmonary collateral arteries, which serve as an additive, or the only source of blood supply to the pulmonary arterial vasculature. These collaterals are usually seen in association with cyanotic congenital heart disease such as pulmonary atresia and tetralogy of fallot. Abstract P1691 Figure. Sequential RVOTO


2021 ◽  
pp. 1-9
Author(s):  
Adeolu Banjoko ◽  
Golnoush Seyedzenouzi ◽  
James Ashton ◽  
Fatemeh Hedayat ◽  
Natalia N. Smith ◽  
...  

Abstract Surgical repair of Tetralogy of Fallot has excellent outcomes, with over 90% of patients alive at 30 years. The ideal time for surgical repair is between 3 and 11 months of age. However, the symptomatic neonate with Tetralogy of Fallot may require earlier intervention: either a palliative intervention (right ventricular outflow tract stent, ductal stent, balloon pulmonary valvuloplasty, or Blalock-Taussig shunt) followed by a surgical repair later on, or a complete surgical repair in the neonatal period. Indications for palliation include prematurity, complex anatomy, small pulmonary artery size, and comorbidities. Given that outcomes after right ventricular outflow tract stent palliation are particularly promising – there is low mortality and morbidity, and consistently increased oxygen saturations and increased pulmonary artery z-scores – it is now considered the first-line palliative option. Disadvantages of right ventricular outflow tract stenting include increased cardiopulmonary bypass time at later repair and the stent preventing pulmonary valve preservation. However, neonatal surgical repair is associated with increased short-term complications and hospital length of stay compared to staged repair. Both staged repair and primary repair appear to have similar long-term mortality and morbidity, but more evidence is needed assessing long-term outcomes for right ventricular outflow tract stent palliation patients.


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