Abstract
Background
D-Transposition of the great arteries (d-TGA) is characterized by the aorta positioned above the right ventricle and the pulmonary artery above the left ventricle. Acute pulmonary ...artery dissection (PAD) is a rare and often lethal condition. We present a case report of acute PAD in an adult with d-TGA and pulmonary hypertension.
Case summary
A 49-year-old male with history of d-TGA palliated with an atrial switch (Mustard) operation, pulmonary venous baffle stenosis treated percutaneously, chronic pulmonary hypertension (mixed group 1 and 2), and severe dilatation of pulmonary arteries (pulmonary trunk of 75 mm) presented to the emergency department with chest pain and acute respiratory failure. Blood pressure was 106/78 mmHg, heart rate 93 b.p.m., and oxygen saturation 88% on room air. A computed tomography (CT) scan showed acute right PAD. He was not considered suitable for surgery nor percutaneous procedure. Epoprostenol was initiated to reduce parietal stress, but after initial stabilization, pulmonary venous stent baffle gradient increased and acute pulmonary oedema occurred. Epoprostenol was withdrawn, and furosemide was initiated, with good clinical response. A follow-up CT scan showed dissection morphological stabilization and false lumen thrombosis, and the patient was discharged.
Discussion
Pulmonary hypertension and previous pulmonary artery dilatation are reported as the main underlying conditions leading to PAD. No previous cases of PAD are described in patients with history of d-TGA and atrial switch procedure. Evidence regarding the best treatment of PAD is lacking, but it seems reasonable to reduce parietal stress using pulmonary vasodilators. Nevertheless, in patients with post-capillary pulmonary hypertension, pulmonary vasodilatation may cause important pulmonary congestion.
To describe the aortic-related risks associated with pregnancy in women with bicuspid aortic valve (BAV) and to evaluate changes in aortic diameter in pregnancy.
Prospective observational study of ...patients with BAV from a single-site registry of pregnant women with structural heart disease between 2013 and 2020. Cardiac, obstetric and neonatal outcomes were studied. An assessment of aortic dimensions was performed during pregnancy by two-dimensional echocardiography. Aortic diameters were measured at the annulus, root, sinotubular junction and maximum ascending aorta diameter, and the largest diameter was used. Measurements of the aorta were made using the end-diastolic leading edge-to-leading edge convention.
Forty-three women (32.9 years, IQR 29.6-35.3) with BAV were included: 9 (20.9%) had repaired aortic coarctation; 23 (53.5%) had moderate or severe aortic valve disease; 5 (11.6%) had a bioprosthetic aortic valve; and 2 (4.7%) had a mechanical prosthetic aortic valve. Twenty (47.0%) were nulliparous. The mean aortic diameter in the first trimester was 38.5 (SD 4.9) mm, and that in the third trimester was 38.4 (SD 4.8) mm. Forty (93.0%) women had an aortic diameter of <45 mm; 3 (7.0%) had 45-50 mm; and none had >50 mm. Three women (6.9%) with BAV presented cardiovascular complications during pregnancy or the postpartum period (two prosthetic thrombosis and one heart failure). No aortic complications were reported. There was a small but significant increase in aortic diameter during pregnancy (third trimester vs first trimester, 0.52 (SD 1.08) mm; p=0.03). Obstetric complications appeared in seven (16.3%) of pregnancies, and there were no maternal deaths. Vaginal non-instrumental delivery was performed in 21 (51.2%) out of 41 cases. There were no neonatal deaths, and the mean newborn weight was 3130 g (95% CI 2652 to 3380).
Pregnancy in BAV women had a low rate of cardiac complications with no aortic complications observed in a small study group. Neither aortic dissection nor need for aortic surgery was reported. A low but significant aortic growth was observed during pregnancy. Although requiring follow-up, the risk of aortic complications in pregnant women with BAV and aortic diameters of <45 mm at baseline is low.
To describe long-term survival and cardiovascular events in adult patients with single ventricle physiology (SVP) without Fontan palliation, focusing on predictors of mortality and comparing groups ...according to their cardiovascular physiology.
Multicentre observational and retrospective study including adult patients with SVP without Fontan palliation since their first adult clinic visit. The cohort was subdivided into 3 groups: Eisenmenger, restricted pulmonary flow, and aortopulmonary shunt. Death was considered as the main end point. Other clinical outcomes occurring during follow-up were considered as secondary end points.
A total of 146 patients, mean age 32.5 ± 11.1 years, were analysed. Over a mean follow-up of 7.3 ± 4.1 years, 33 patients (22.6%) died. Survival was 86% and 74% at 5 and 10 years, respectively. Right ventricular morphology was not associated with higher mortality. Four variables at baseline were related to a higher mortality: at least moderate atrioventricular valve regurgitation, platelet count < 150 × 103/mm3, GFR < 60 mL/min/1.73 m2, and QRS > 120 ms). A total of 34.2% of patients were admitted to the hospital due to heart failure, and 7.5% received a heart transplant. Other cardiovascular outcomes were also frequent: atrial arrhythmias in 19.2%, stroke in 15.1%, and pacemaker/implantable cardioverter-defibrillator in 6.2%/2.7%.
Adult patients with SVP who had not undergone Fontan exhibit a high mortality rate and frequent major cardiovascular events. At least moderate atrioventricular valve regurgitation, thrombocytopenia, renal dysfunction, and QRS duration > 120 ms at baseline visit allow identification of a cohort of patients at higher risk of mortality.
Décrire la survie à long terme et les événements cardiovasculaires chez les patients adultes présentant une physiologie à ventricule unique (PVU) sans intervention de Fontan, en se concentrant sur les prédicteurs de mortalité et en comparant les groupes en fonction de leur physiologie cardiovasculaire.
Étude observationnelle et rétrospective, multicentrique, incluant des patients adultes atteints de PVU sans intervention de Fontan depuis leur première visite en clinique adulte. La cohorte a été subdivisée en trois groupes : syndrome d'Eisenmenger, flux pulmonaire restreint ou fenêtre aorto-pulmonaire. Le décès a été considéré comme le critère principal d'évaluation. Les autres observations cliniques survenues au cours du suivi ont été considérées comme des critères secondaires.
Un total de 146 patients, d'un âge moyen de 32,5 ± 11,1 ans, a été considéré pour l’analyse. Sur un suivi moyen de 7,3 ± 4,1 ans, 33 patients (22,6 %) sont décédés. La survie était de 86 % et 74 % à 5 et 10 ans, respectivement. La morphologie du ventricule droit n'était pas associée à une mortalité plus élevée. Quatre variables initiales étaient liées à une mortalité plus élevée : régurgitation au moins modérée de la valve auriculo-ventriculaire, numération plaquettaire < 150 × 103/mm3, DFG < 60 ml/min/1,73 m2 et QRS > 120 ms). Au total, 34,2 % des patients ont été admis à l'hôpital en raison d'une insuffisance cardiaque, et 7,5 % ont reçu une transplantation cardiaque. D'autres conséquences cardiovasculaires étaient également fréquentes : arythmies auriculaires dans 19,2 % des cas, accident vasculaire cérébral dans 15,1 % des cas et stimulateur cardiaque/ défibrillateur cardioverteur implantable dans 6,2 % / 2,7 % des cas.
Les patients adultes atteints de PVU qui n'ont pas subi d'intervention de Fontan présentent un taux de mortalité élevé et des événements cardiovasculaires majeurs fréquents. Une régurgitation au moins modérée de la valve auriculo-ventriculaire, une thrombocytopénie, une dysfonction rénale et une durée du QRS > 120 ms lors de la visite initiale permettent de distinguer une cohorte de patients présentant un risque de mortalité plus élevé.
BackgroundD-Transposition of the great arteries (d-TGA) is characterized by the aorta positioned above the right ventricle and the pulmonary artery above the left ventricle. Acute pulmonary artery ...dissection (PAD) is a rare and often lethal condition. We present a case report of acute PAD in an adult with d-TGA and pulmonary hypertension.Case summaryA 49-year-old male with history of d-TGA palliated with an atrial switch (Mustard) operation, pulmonary venous baffle stenosis treated percutaneously, chronic pulmonary hypertension (mixed group 1 and 2), and severe dilatation of pulmonary arteries (pulmonary trunk of 75 mm) presented to the emergency department with chest pain and acute respiratory failure. Blood pressure was 106/78 mmHg, heart rate 93 b.p.m., and oxygen saturation 88% on room air. A computed tomography (CT) scan showed acute right PAD. He was not considered suitable for surgery nor percutaneous procedure. Epoprostenol was initiated to reduce parietal stress, but after initial stabilization, pulmonary venous stent baffle gradient increased and acute pulmonary oedema occurred. Epoprostenol was withdrawn, and furosemide was initiated, with good clinical response. A follow-up CT scan showed dissection morphological stabilization and false lumen thrombosis, and the patient was discharged.DiscussionPulmonary hypertension and previous pulmonary artery dilatation are reported as the main underlying conditions leading to PAD. No previous cases of PAD are described in patients with history of d-TGA and atrial switch procedure. Evidence regarding the best treatment of PAD is lacking, but it seems reasonable to reduce parietal stress using pulmonary vasodilators. Nevertheless, in patients with post-capillary pulmonary hypertension, pulmonary vasodilatation may cause important pulmonary congestion.
Peritoneal metastasis in patients with locally advanced colon cancer (T4 stage) is estimated to recur at a rate of approximately 25% at 3 years from surgical resection and is associated with poor ...prognosis. There is controversy regarding the clinical benefit of prophylactic hyperthermic intraperitoneal chemotherapy (HIPEC) in these patients.
To assess the efficacy and safety of intraoperative HIPEC in patients with locally advanced colon cancer.
This open-label, phase 3 randomized clinical trial was conducted in 17 Spanish centers from November 15, 2015, to March 9, 2021. Enrolled patients were aged 18 to 75 years with locally advanced primary colon cancer diagnosed preoperatively (cT4N02M0).
Patients were randomly assigned 1:1 to receive cytoreduction plus HIPEC with mitomycin C (30 mg/m2 over 60 minutes; investigational group) or cytoreduction alone (comparator group), both followed by systemic adjuvant chemotherapy. Randomization of the intention-to-treat population was done via a web-based system, with stratification by treatment center and sex.
The primary outcome was 3-year locoregional control (LC) rate, defined as the proportion of patients without peritoneal disease recurrence analyzed by intention to treat. Secondary end points were disease-free survival, overall survival, morbidity, and rate of toxic effects.
A total of 184 patients were recruited and randomized (investigational group, n = 89; comparator group, n = 95). The mean (SD) age was 61.5 (9.2) years, and 111 (60.3%) were male. Median duration of follow-up was 36 months (IQR, 27-36 months). Demographic and clinical characteristics were similar between groups. The 3-year LC rate was higher in the investigational group (97.6%) than in the comparator group (87.6%) (log-rank P = .03; hazard ratio HR, 0.21; 95% CI, 0.05-0.95). No differences were observed in disease-free survival (investigational, 81.2%; comparator, 78.0%; log-rank P = .22; HR, 0.71; 95% CI, 0.41-1.22) or overall survival (investigational, 91.7%; comparator, 92.9%; log-rank P = .68; HR, 0.79; 95% CI, 0.26-2.37). The definitive subgroup with pT4 disease showed a pronounced benefit in 3-year LC rate after investigational treatment (investigational: 98.3%; comparator: 82.1%; log-rank P = .003; HR, 0.09; 95% CI, 0.01-0.70). No differences in morbidity or toxic effects between groups were observed.
In this randomized clinical trial, the addition of HIPEC to complete surgical resection for locally advanced colon cancer improved the 3-year LC rate compared with surgery alone. This approach should be considered for patients with locally advanced colorectal cancer.
ClinicalTrials.gov Identifier: NCT02614534.
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