Surgical treatment of isolated unilateral absence of pulmonary artery with pulmonary hypertension

2021 ◽  
pp. 1-2
Author(s):  
Moyu Hasegawa ◽  
Shigemitsu Iwai ◽  
Kazuhiko Ishimaru

Abstract A one-and-a-half-month-old patient with isolated unilateral absence of the right proximal pulmonary artery with prominent left pulmonary hypertension was diagnosed using CT. Medication therapy was initiated first. Left pulmonary artery pressure decreased after the initiation of medication therapy, and single-stage reconstruction of the right pulmonary artery was performed. The patient is asymptomatic 14 months postoperatively.

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
J Grade Santos ◽  
F Ferreira ◽  
M Loureiro ◽  
A Almeida ◽  
A Pereira ◽  
...  

Abstract A 32 year old female patient, with a medical history of an ill-characterized Pulmonary Arterial Hypertension associated with congenital heart disease, lost in the follow up with no medical therapy, attended an emergency department for a gynecological hemorrhage at 16 weeks of pregnancy. Due to high maternity mortality risk, informed consent was obtained, and termination of pregnancy was performed. She was then referred to our pulmonary hypertension center. At our center she had complains of fatigue with moderate intensity exertion, classified in a class II of the World Health Organization (WHO) classification, but was otherwise asymptomatic, with no history of dyspnea, angina or syncope. There was allusion to a self-limited episode of hemoptysis in the past. On physical examination she had an increased pulmonary component of the second heart sound, continuous heart murmur in left sternal border and no cyanosis (O2 peripheral saturation in the upper and lower limbs of 99% at room air). The performed echocardiograms (both transthoracic and transesophageal) showed an estimated systolic pulmonary artery pressure of 120 mmHg with severe right ventricular hypertrophy and systolic dysfunction. There was dilatation of the trunk and right pulmonary artery. The left pulmonary artery was not seen. Biochemical evaluation and viral serologies were unremarkable. The pulmonary function tests and the arterial blood gases were normal. Cardiac MRI demonstrated the presence of a right aortic arch and a right patent arterial duct. An anomalous origin of the left pulmonary artery from the ascending thoracic aorta could be noted. Associated congenital cardiac defects were excluded. A right heart catheterization confirmed the presence of severe pulmonary hypertension with mean pulmonary artery pressure of 86 mmHg and Pulmonary vascular resistance of 11 Wood Units. A large persistent arterial duct to the right pulmonary artery was confirmed with persistent left to right shunt. The left pulmonary artery was visualized when injection was performed in the aortic root. Coronary arteries were normally implanted. The patient was started on Sildenafil and Bosentan (later replaced by Macitentan due to hepatic toxicity). After 3 years of follow up, there was an improvement in symptoms and in the 6 minutes walking test, remaining in a low risk category and on a WHO class I. This case reports a very rare congenital abnormality identified in an adult patient. Despite the complex anatomy and severe pulmonary hypertension, the patient is reasonably well under medical therapy and close follow up. Abstract P1728 Figure. Cardiac MRI Cine Sequences


2020 ◽  
Vol 9 (3) ◽  
pp. e44932309
Author(s):  
Tamyris Beluque ◽  
Bruna Natali da Costa ◽  
Beatriz de Carvalho Pato Vila ◽  
Vinícius Bentivóglio Costa Silva ◽  
Marlos Gonçalves Sousa

This paper aims to perform an analysis of the indirect methods of pulmonary artery pressure assessment based on the scientific literature. Through bibliographic survey, scientific articles were selected according to their relevance to the theme.The assessment of pulmonary artery pressure allows to infer whether the individual has pulmonary arterial hypertension (PAH), which is associated with a poor prognosis in dogs diagnosed with mitral valve degeneration. The standard examination is pulmonary artery catheterization, but this method has not been used in routine veterinary medicine because the clinical status of the animal usually does not allow sedation and it is a costly procedure. The diagnosis of PAH in small animals through Doppler echocardiography can be obtained by evaluating the regurgitation of the tricuspid or pulmonary valve, subjective characteristics and measurement of the acceleration time (AT) and the acceleration time-to-ejection time ratio of pulmonary artery (AT:ET), pulmonary artery-to-pulmonary vein ratio, distensibility of the right pulmonary artery (RAPD), and assessment of right ventricular function, especially TEI index and strain. The aim of this review was to demonstrate the main echocardiographic techniques to estimate PAH, with the intention of contributing to the cardiologist in the diagnosis of PAH. All techniques have positive and negative factors however, it is important to emphasize that there is no ideal technique, and the most assertive way to arrive at the diagnosis is to associate the different techniques.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
KIRILL Malikov ◽  
MARINA Kirichkova ◽  
MARIA Simakova ◽  
NARECK Marukyan ◽  
OLGA Moiseeva

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Chronic thromboembolic pulmonary hypertension (CTEPH) leads to a progressive increase in pulmonary vascular resistance (PVR) and pulmonary artery pressure (PAP) with the development of severe dysfunction of the right heart and heart failure. Mortality for three years with an average pressure in the pulmonary artery (PA) of more than 50 mmHg is more than 90%. Balloon pulmonary angioplasty (BPA) has a significant advantage over other methods of surgical treatment, but it requires the determination of additional non-invasive markers of effectiveness. Transthoracic echocardiography (TTE) remains the main method for assessing the morphology and function of the heart. Purpose Compare different indicators reflecting the severity of CTEPH with TTE indicators before and after BPA. To evaluate the effectiveness of using BPA for the treatment of patients with CTEPH using routine TTE and speckle tracking mode. Materials and methods For 18 months 30 patients without concomitant cardiovascular pathology were subjected to several BPA sessions. Before treatment, 50% of patients belonged to the 3 CTEPH functional class (FC), 40% to 2 FC, 10% to 1 FC. The average number of sessions was 4.7 ± 1.3. Before the first BPA and after the last, all the patients were performed: six-minute walk test (6MWT, metres), Borg scale (in points), test for NT-proBNP (pg/ml); TTE with assessment of the right ventricle (RV) and left ventricle (LV) including areas of the right atrium (aRA, cm2), mean pulmonary artery pressure (PUPM,mmHg),RV free wall strain (GLSFW, %), RV free wall strain rate (GLSRFW, sm/sec), RV free wall postsystolic shortening (PSSFW, %), tricuspid annular plane systolic excursion (TAPSE, sm), tricuspid annulus systolic velocity (TASV, sm/sec). Results. Before the first BPA session, the 6MWT in the patient group averaged 315.9 ± 9.08 metres, after - 439.5 ± 11.45 m; the Borg from 5.4 ± 0.94 points decreased to 4 ± 1.01 points; NT-proBNP before the treatment was 1513 ± 13.01 pg/ml, after - 171 ± 6.09; according to TTE the ratio of RV/ LV before and after treatment was 1.31 ± 0.02 and 0.97 ± 0.04; aRA was 29.3 ± 4.87 and 22.3 ± 3.53 cm2; basal RV - 52 ± 5.11 and 44 ± 7.26 mm; PUPM decreased from 76.6 ± 7.65 to 31.3 ± 3.78 mmHg; GLSFW from -14.69 ± 2.33 came to 17.5 ± 3.45 %; GLSRFW with -0.9 ± 0.09 to -1.7 ± 0.11 cm/sec; TAPSE from 16.7 ± 1.87 to 18.2 ± 2.34 cm; TASV from 10.11 ± 1.45 to 12.25 ± 1.98 cm/s, PSSFW before treatment was -18.4 ± 1.2%, after treatment in 66% of patients disappeared, in 34% became an average of 17.4 ± 0.9% The distribution of STEPH FC has also changed. Conclusion. BPA leads to an improvement in the tolerance of physical activity, clinical indicators, and parameters of central hemodynamics in the pulmonary circulation, evaluated according to direct manometry, and leads to reverse remodeling of the RV in the long term. Performing a staged BPA leads to an improvement in the functional parameters of contractility of the RV.


Biomedicines ◽  
2021 ◽  
Vol 9 (9) ◽  
pp. 1212
Author(s):  
Fabian Mueller-Graf ◽  
Jonas Merz ◽  
Tim Bandorf ◽  
Chiara Albus ◽  
Maike Henkel ◽  
...  

For the non-invasive assessment of pulmonary artery pressure (PAP), surrogates like pulse wave transit time (PWTT) have been proposed. The aim of this study was to invasively validate for which kind of PAP (systolic, mean, or diastolic) PWTT is the best surrogate parameter. To assess both PWTT and PAP in six healthy pigs, two pulmonary artery Mikro-Tip™ catheters were inserted into the pulmonary vasculature at a fixed distance: one in the pulmonary artery trunk, and a second one in a distal segment of the pulmonary artery. PAP was raised using the thromboxane A2 analogue U46619 (TXA) and by hypoxic vasoconstriction. There was a negative linear correlation between PWTT and systolic PAP (r = 0.742), mean PAP (r = 0.712) and diastolic PAP (r = 0.609) under TXA. During hypoxic vasoconstriction, the correlation coefficients for systolic, mean, and diastolic PAP were consistently higher than for TXA-induced pulmonary hypertension (r = 0.809, 0.778 and 0.734, respectively). Estimation of sPAP, mPAP, and dPAP using PWTT is feasible, nevertheless slightly better correlation coefficients were detected for sPAP compared to dPAP. In this study we establish the physiological basis for future methods to obtain PAP by non-invasively measured PWTT.


2013 ◽  
Vol 114 (3) ◽  
pp. 154-161 ◽  
Author(s):  
Mehmet Demir ◽  
U. Uyan ◽  
S. Keçeoçlu ◽  
C. Demir

Vitamin D deficiency actives renin-angiotensin-aldosterone system (RAAS) which affects cardiovascular system. Activation of RAAS is associated with pulmonary hypertension (PHT). Relation between vitamin D deficiency and PHT could be therefore suggested. In  our study we compared pulmonary artery pressure between vitamin D deficiency and control groups. 115 consecutive patients (average age: 61.86 ± 5.86) who have detected very low vitamin D (vitamin D levels < 10 ng/ml) were enrolled. 117 age matched persons (average age: 61.74 ± 5.99) were selected as the control group. All groups underwent transthoracic echocardiography. Routine biochemical measurement of 25-OH vitamin D and parathormon (PTH) levels were performed. Baseline characteristics of the study groups were comparable. Systolic pulmonary artery pressure (SPAP) of patients in  the low vitamin D group was higher than the control groups. As a  result our study, a  relation between vitamin D deficiency and pulmonary artery hypertension was revealed.


1995 ◽  
Vol 117 (2) ◽  
pp. 237-241
Author(s):  
H. Katayama ◽  
G. W. Henry ◽  
C. L. Lucas ◽  
B. Ha ◽  
J. I. Ferreiro ◽  
...  

We studied the detailed profiles of blood flow in the right and left pulmonary arteries using 20 MHz pulsed Doppler ultrasound equipment in a lamb model. Fourteen lambs aged four to six weeks were selected. In six lambs, monocrotaline pyrrole was injected parenterally to create pulmonary hypertension (PH group). Eight other lambs served as unaltered controls (control group). The blood flow velocities were sampled in 1mm increments along the anterior—posterior axis of the branch arteries. The maximum velocity of the forward flow in the left pulmonary artery was higher than that in the right pulmonary artery in the control group (71.7 ± 15.9cm/s vs 60.2 ± 13.5; p < 0.05). The fastest backward flow was located at the posterior position of the vessel in the right pulmonary artery in the control group. No significant bias in location was shown in the left pulmonary artery. Using indices of P90, acceleration time, P90*AcT, the velocity waveforms in the PH group were compared with those in the control group. In the left pulmonary artery, every index in the control group showed a significantly greater value that in the PH group. On the other hand, no significant differences were found between either group in the right pulmonary artery.


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