-
Medicine Jun 2024Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare but serious complication in patients with malignancy; its main manifestation includes acute pulmonary... (Review)
Review
RATIONALE
Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare but serious complication in patients with malignancy; its main manifestation includes acute pulmonary hypertension with severe respiratory distress. More than 200 cases have been reported since it was first identified in 1990. PTTM accounts for approximately 0.9% to 3.3% of deaths due to malignancy, but only a minority of patients are diagnosed ante-mortem, with most patients having a definitive diagnosis after autopsy.
PATIENT CONCERNS
Two middle-aged women both died within a short period of time due to progressive dyspnea and severe pulmonary hypertension.
DIAGNOSES
One patient was definitively confirmed as a gastrointestinal malignant tumor by liver puncture biopsy pathology. Ultimately, the clinical diagnosis was pulmonary tumor thrombotic microangiopathy.
INTERVENTIONS
The patient was treated symptomatically with oxygen, diuresis, and anticoagulation, while a liver puncture was perfected to clarify the cause.
OUTCOMES
Two cases of middle-aged female patients with rapidly progressive pulmonary hypertension and respiratory failure resulted in death with malignant neoplasm.
LESSONS
PTTM has a rapid onset and a high morbidity and mortality rate. Our clinicians need to be more aware of the need for timely diagnosis through a targeted clinical approach, leading to more targeted treatment and a better prognosis.
Topics: Humans; Female; Thrombotic Microangiopathies; Middle Aged; Fatal Outcome; Hypertension, Pulmonary; Gastrointestinal Neoplasms; Lung Neoplasms
PubMed: 38941435
DOI: 10.1097/MD.0000000000038618 -
Medicine Jun 2024Complete dislodgement of a mechanical valve is extremely uncommon as a long-term issue after getting a substitute mitral valve, and this report details an incident of...
RATIONALE
Complete dislodgement of a mechanical valve is extremely uncommon as a long-term issue after getting a substitute mitral valve, and this report details an incident of complete detachment of a mechanical valve.
PATIENT CONCERNS
A 50-year-old woman, who underwent mitral mechanical valve replacement 2 decades earlier at another facility, was urgently admitted due to sudden cardiogenic shock.
DIAGNOSES
Transthoracic echocardiograms revealed severe malfunction of the mitral valve prosthesis, characterized by significant mitral regurgitation and moderate pulmonary hypertension. Following the insertion of extracorporeal membrane oxygenation and an intra-aortic balloon pump, the hemodynamics stabilized. Coronary angiography displayed the prosthetic mitral valve ring and leaflet floating in the left atrium, as confirmed by preoperative real-time 3-dimensional transesophageal echocardiography. A complete separation of the prosthetic ring and leaflet from the suture ring was observed.
INTERVENTIONS
The patient promptly underwent bioprosthetic mitral valve replacement.
OUTCOMES
The patient's postoperative course was uneventful, leading to discharge in good condition.
LESSONS
A crucial aspect is comprehending the structure of the prosthetic valve itself. The use of transthoracic echocardiography and real-time 3-dimensional transesophageal echocardiography provides additional structural and functional details, enhancing support for potential life-saving interventions. Echocardiography plays a significant role in evaluating the morphology and function of prosthetic valves.
Topics: Humans; Female; Middle Aged; Heart Valve Prosthesis; Mitral Valve Insufficiency; Prosthesis Failure; Mitral Valve; Heart Valve Prosthesis Implantation; Echocardiography, Transesophageal; Shock, Cardiogenic; Echocardiography, Three-Dimensional
PubMed: 38941392
DOI: 10.1097/MD.0000000000038612 -
JACC. Advances Jan 2024Hypertensive disorders of pregnancy (HDP) can be classified into gestational hypertension, preeclampsia (PRE), and chronic hypertension with superimposed preeclampsia...
BACKGROUND
Hypertensive disorders of pregnancy (HDP) can be classified into gestational hypertension, preeclampsia (PRE), and chronic hypertension with superimposed preeclampsia (SPE).
OBJECTIVES
The purpose of this study was to retrospectively examine the echocardiographic differences in biventricular structure and function in 3 HDP groups of women in comparison to normotensive pregnant controls.
METHODS
Women with an echocardiogram during or within the first year of pregnancy were identified within our integrated health network. Exclusion criteria included age <18 years, diagnosis of pulmonary embolism, malignancy, autoimmune disease, and structural heart disease.
RESULTS
We identified a total of 706 subjects (cases: n = 427, normotensive controls: n = 279). Cases were divided into 3 groups: gestational hypertension (n = 57), PRE (n = 291), and SPE (n = 79). In adjusted analyses, echocardiographic parameters demonstrated a graded difference in left ventricular (LV) mass index, relative wall thickness, mitral inflow E, mitral inflow A, septal e', lateral e', E/e', left atrial volume index, tricuspid velocity, and lateral e' velocities with the most profound findings noted in the SPE group. Specifically, adjusted LV mass index (adjusted β = 14.45, 95% CI: 9.00-19.90) and E/e' (adjusted β = 2.97, 95% CI: 2.27-3.68) was highest in the SPE group in comparison to controls ( < 0.001).
CONCLUSIONS
LV remodeling and diastolic filling abnormalities are more common in HDP and are most evident in SPE and PRE. Echocardiography during or immediately after pregnancy may be useful in these high-risk women to identify these abnormalities. The long-term implications of these echocardiographic abnormalities require further study.
PubMed: 38939814
DOI: 10.1016/j.jacadv.2023.100725 -
Admission Total Leukocyte Count as a Predictor of Mortality in Cardiac Intensive Care Unit Patients.JACC. Advances Jan 2024Inflammation is a sequela of cardiovascular critical illness and a risk factor for mortality.
BACKGROUND
Inflammation is a sequela of cardiovascular critical illness and a risk factor for mortality.
OBJECTIVES
This study aimed to evaluate the association between white blood cell count (WBC) and mortality in a broad population of patients admitted to the cardiac intensive care unit (CICU).
METHODS
This retrospective cohort study included patients admitted to the Mayo Clinic CICU between 2007 and 2018. We analyzed WBC as a continuous variable and then categorized WBC as low (<4.0 × 10/mL), normal (≥4.0 to <11.0 × 10/mL), high (≥11.0 to <22.0 × 10/mL), or very high (≥22.0 × 10/mL). The association between WBC and in-hospital mortality was evaluated using multivariable logistic regression and random forest models.
RESULTS
We included 11,699 patients with a median age of 69.3 years (37.6% females). Median WBC was 9.6 (IQR: 7.4-12.7). Mortality was higher in the low (10.5%), high (12.0%), and very high (33.3%) WBC groups relative to the normal WBC group (5.3%). A rising WBC was incrementally associated with higher in-hospital mortality after adjustment (AICc adjusted OR: 1.03 [95% CI: 1.02-1.04] per 1 × 10 increase in WBC). After adjustment, only the high (AICc adjusted OR: 1.37 [95% CI: 1.15-1.64]) and very high (AICc adjusted OR: 1.99 [1.47-2.71]) WBC groups remained associated with increased risk of in-hospital mortality.
CONCLUSIONS
Leukocytosis is associated with an increased mortality risk in a diverse cohort of CICU patients. This readily available marker of systemic inflammation may be useful for risk stratification within the increasingly complex CICU patient population.
PubMed: 38939813
DOI: 10.1016/j.jacadv.2023.100757 -
JACC. Advances Apr 2024
PubMed: 38939683
DOI: 10.1016/j.jacadv.2024.100891 -
JACC. Advances Apr 2024A treatment strategy for congenital heart defects with moderate to severe pulmonary arterial hypertension (PAH) has not been established.
BACKGROUND
A treatment strategy for congenital heart defects with moderate to severe pulmonary arterial hypertension (PAH) has not been established.
OBJECTIVES
The purpose of this study was to identify patients in whom a treat and repair strategy was considered and to examine pretreatment variables associated with successful defect repair.
METHODS
Patients with atrial or ventricular septal defect and PAH (pulmonary vascular resistance [PVR] ≥ 5 Wood units) eligible for the treat and repair strategy were included. Hemodynamics among pretreatment, pre-repair, and post-defect repair were compared. Clinical outcomes in patients with or without defect repair were also compared. Clinical outcomes included all-cause death, hospitalization for worsening pulmonary hypertension, and lung transplantation.
RESULTS
Among 25 eligible for the treat and repair strategy, 20 underwent successful repair (repaired group) and 5 did not have a repair (unrepaired group). In the repaired group, PVR significantly decreased from 9.6 ± 2.6 WU at pretreatment to 5.0 ± 3.4 pre-repair (ß coefficient -4.6 [95% CI: -5.9 to -3.3]). The pulmonary to systemic blood flow ratio (Qp/Qs) increased from 1.5 ± 0.6 at pretreatment to 2.4 ± 1.3 pre-repair (ß coefficient 0.9 [95% CI: 0.4-1.38]). In the unrepaired group, pretreatment PVR decreased with treatment; however, PVR remained elevated. Qp/Qs did not change between pretreatment and post-treatment. The repaired group had a better prognosis than the unrepaired group (HR 0.092 [95% CI: 0.009-0.905]). Pretreatment mean pulmonary artery pressure, PVR, Qp/Qs, and arterial oxygen saturations were associated with undergoing defect repair.
CONCLUSIONS
In this small cohort, a treat and repair strategy was successfully used in a significant proportion of the patients with congenital heart defects with moderate to severe PAH.
PubMed: 38939653
DOI: 10.1016/j.jacadv.2024.100887 -
JACC. Advances Sep 2023Most risk prediction models are confined to specific medical conditions, thus limiting their application to general medical populations.
BACKGROUND
Most risk prediction models are confined to specific medical conditions, thus limiting their application to general medical populations.
OBJECTIVES
The MARKER-HF (Machine learning Assessment of RisK and EaRly mortality in Heart Failure) risk model was developed in heart failure (HF) patients. We assessed the ability of MARKER-HF to predict 1-year mortality in a large community-based hospital registry database including patients with and without HF.
METHODS
This study included 41,749 consecutive patients who underwent echocardiography in a tertiary referral hospital (4,640 patients with and 37,109 without HF). Patients without HF were further subdivided into those with (n = 22,946) and without cardiovascular disease (n = 14,163) and also into cohorts based on recent acute coronary syndrome or history of atrial fibrillation, chronic obstructive pulmonary disease, chronic kidney disease, diabetes mellitus, hypertension, or malignancy.
RESULTS
The median age of the 41,749 patients was 65 years, and 56.2% were male. The receiver operated area under the curves for MARKER-HF prediction of 1-year mortality of patients with HF was 0.729 (95% CI: 0.706-0.752) and for patients without HF was 0.770 (95% CI: 0.760-0.780). MARKER-HF prediction of mortality was consistent across subgroups with and without cardiovascular disease and in patients diagnosed with acute coronary syndrome, atrial fibrillation, chronic obstructive pulmonary disease, chronic kidney disease, diabetes mellitus, or hypertension. Patients with malignancy demonstrated higher mortality at a given MARKER-HF score than did patients in the other groups.
CONCLUSIONS
MARKER-HF predicts mortality for patients with HF as well as for patients suffering from a variety of diseases.
PubMed: 38939487
DOI: 10.1016/j.jacadv.2023.100554 -
JACC. Advances Sep 2023Current guidelines recommend concomitant repair of certain non-severe cases of tricuspid regurgitation (TR) in patients undergoing cardiac surgery, but the prognostic...
BACKGROUND
Current guidelines recommend concomitant repair of certain non-severe cases of tricuspid regurgitation (TR) in patients undergoing cardiac surgery, but the prognostic relevance and postsurgical impact of the TR remain uncertain.
OBJECTIVES
The purpose of this study was to determine the prognostic impact of functional TR in patients undergoing diverse cardiac surgeries and to examine the effect-modifying role of patient characteristics in patients in whom TR confers a greater risk of adverse outcomes.
METHODS
Patients undergoing coronary artery bypass, aortic, and mitral valve surgery were included. Patients with severe TR, organic tricuspid valve pathology, undergoing tricuspid valve surgery or without a recent preoperative echocardiogram were excluded. Clinical variables were extracted from the Society of Thoracic Surgeons Adult Cardiac Surgery Database. An independent cohort was used for external validation.
RESULTS
Of 2,119 patients (mean age 67.4 years; 29% females), TR severity was moderate in 185 (9%), mild in 636 (30%), trivial in 1,126 (53%), and absent in 172 (8%). There were 238 deaths during the median follow-up period of 2.6 years. After adjusting for relevant factors, moderate TR was found to be independently associated with mid-term mortality (HR: 2.58; 95% CI: 1.22-5.47) and with in-hospital mortality or major morbidity (OR: 3.18; 95% CI: 1.37-7.42). The association between TR and mortality was apparent when preoperative pulmonary artery systolic pressure was <40 mm Hg but not ≥40 mm Hg ( for interaction = 0.036).
CONCLUSIONS
In this diverse cohort of contemporary cardiac surgery patients, moderate functional TR was associated with increased mortality and major morbidity, particularly in the absence of pulmonary hypertension.
PubMed: 38939486
DOI: 10.1016/j.jacadv.2023.100551 -
JACC. Advances Aug 2023Detection of heart failure with preserved ejection fraction (HFpEF) involves integration of multiple imaging and clinical features which are often discordant or...
BACKGROUND
Detection of heart failure with preserved ejection fraction (HFpEF) involves integration of multiple imaging and clinical features which are often discordant or indeterminate.
OBJECTIVES
The authors applied artificial intelligence (AI) to analyze a single apical 4-chamber transthoracic echocardiogram video clip to detect HFpEF.
METHODS
A 3-dimensional convolutional neural network was developed and trained on apical 4-chamber video clips to classify patients with HFpEF (diagnosis of heart failure, ejection fraction ≥50%, and echocardiographic evidence of increased filling pressure; cases) vs without HFpEF (ejection fraction ≥50%, no diagnosis of heart failure, normal filling pressure; controls). Model outputs were classified as HFpEF, no HFpEF, or nondiagnostic (high uncertainty). Performance was assessed in an independent multisite data set and compared to previously validated clinical scores.
RESULTS
Training and validation included 2,971 cases and 3,785 controls (validation holdout, 16.8% patients), and demonstrated excellent discrimination (area under receiver-operating characteristic curve: 0.97 [95% CI: 0.96-0.97] and 0.95 [95% CI: 0.93-0.96] in training and validation, respectively). In independent testing (646 cases, 638 controls), 94 (7.3%) were nondiagnostic; sensitivity (87.8%; 95% CI: 84.5%-90.9%) and specificity (81.9%; 95% CI: 78.2%-85.6%) were maintained in clinically relevant subgroups, with high repeatability and reproducibility. Of 701 and 776 indeterminate outputs from the Heart Failure Association-Pretest Assessment, Echocardiographic and Natriuretic Peptide Score, Functional Testing (HFA-PEFF), and Final Etiology and Heavy, Hypertensive, Atrial Fibrillation, Pulmonary Hypertension, Elder, and Filling Pressure (H2FPEF) scores, the AI HFpEF model correctly reclassified 73.5% and 73.6%, respectively. During follow-up (median: 2.3 [IQR: 0.5-5.6] years), 444 (34.6%) patients died; mortality was higher in patients classified as HFpEF by AI (HR: 1.9 [95% CI: 1.5-2.4]).
CONCLUSIONS
An AI HFpEF model based on a single, routinely acquired echocardiographic video demonstrated excellent discrimination of patients with vs without HFpEF, more often than clinical scores, and identified patients with higher mortality.
PubMed: 38939447
DOI: 10.1016/j.jacadv.2023.100452 -
JACC. Advances Mar 2024Pulmonary hypertension (PH) has been shown to be associated with worse outcomes in patients with aortic regurgitation (AR) in small older studies.
BACKGROUND
Pulmonary hypertension (PH) has been shown to be associated with worse outcomes in patients with aortic regurgitation (AR) in small older studies.
OBJECTIVES
The authors sought to evaluate the prevalence of PH in patients with severe AR, its impact on mortality and symptoms, and regression after aortic valve replacement (AVR).
METHODS
A total of 821 consecutive patients with chronic ≥ moderate-severe AR on echocardiography from 2004 to 2019 were retrospectively analyzed. PH was defined as right ventricular systolic pressure (RVSP) >40 mm Hg on transthoracic echocardiogram (mild-moderate PH: RVSP 40-59 mm Hg, severe PH: RVSP > 60 mm Hg). Clinical and echocardiographic data were extracted from the electronic medical record and echocardiographic reports. The diastolic function and filling pressures were manually assessed and checked, and the left ventricular (LV) volumes were traced by a level 3-trained echocardiographer. The primary objectives were prevalence of PH in patients with ≥ moderate-severe AR, its risk associations and impact on all-cause mortality as the primary outcome. Secondary outcomes were impact of PH on symptoms and change in RVSP at discharge post-AVR. Logistic and Cox proportional hazards regression were used to analyze these outcomes.
RESULTS
The mean age was 61.2 ± 17 years, and 162 (20%) were women. Mild-moderate PH was present in 91 (11%) patients and severe PH in 27 (3%). Larger LV size, elevated LV filling pressures, and ≥ moderate tricuspid regurgitation were associated with PH. During follow-up of 7.3 (6.3-7.9) years, 188 patients died. Compared to those without PH, risk of mortality was higher in mild-moderate PH (adjusted HR: 1.59 (95% CI: 1.07-2.36) ( = 0.021)) and severe PH (adjusted HR: 2.90 (95% CI: 1.63-5.15) ( < 0.001)). Symptoms were also more prevalent in those with PH ( = 0.004). Of 396 patients who underwent AVR during the study period, 57 had PH. AVR similarly improved survival in patients without and with PH ( for interaction = 0.23), and there was regression in RVSP (≥8 mm Hg drop) at discharge post-AVR in 35/57 (61%) patients with PH.
CONCLUSIONS
PH was present in 14% of patients with AR and was associated with higher mortality and symptoms. The survival benefit of AVR was similar in patients without and with PH.
PubMed: 38938846
DOI: 10.1016/j.jacadv.2024.100827