Pediatr Crit Care Med. 2018 Feb 6. doi: 10.1097/PCC.0000000000001464. [Epub ahead of print]

Characteristics of Infants With Congenital Diaphragmatic Hernia Who Need Follow-Up of Pulmonary Hypertension.

Kraemer US^1,2, Leeuwen L¹, Krasemann TB², Wijnen RMH¹, Tibboel D¹, IJsselstijn H¹.

Author information:

1. Intensive Care and Department of Pediatric Surgery, Erasmus MC-Sophia Children’s Hospital, Rotterdam, The Netherlands.
2. Department of Pediatrics, Division of Pediatric Cardiology, Erasmus MC-Sophia Children’s Hospital, Rotterdam, The Netherlands.

Abstract

OBJECTIVES:

Pulmonary hypertension is one of the main causes of mortality and morbidity in patients with congenital diaphragmatic hernia. Currently, it is unknown whether pulmonary hypertension persists or recurs during the first year of life.

DESIGN:

Prospective longitudinal follow-up study.

SETTING:

Tertiary university hospital.

PATIENTS:

Fifty-two congenital diaphragmatic hernia patients admitted between 2010 and 2014.

INTERVENTIONS:

None.

MEASUREMENTS AND MAIN RESULTS:

Pulmonary hypertension was measured using echocardiography and electrocardiography at 6 and 12 months old. Characteristics of patients with persistent pulmonary hypertension were compared with those of patients without persistent pulmonary hypertension. At follow-up, pulmonary hypertension persisted in four patients: at 6 months old, in three patients (patients A-C), and at 12 months old, in two patients (patients C and D). Patients with persistent pulmonary hypertension had a longer duration of mechanical ventilation (median 77 d [interquartile range, 49-181 d] vs median 8 d [interquartile range, 5-15 d]; p = 0.002) and hospital stay (median 331 d [interquartile range, 198-407 d) vs median 33 d (interquartile range, 16-59 d]; p = 0.003) than patients without persistent pulmonary hypertension. The proportion of patients with persistent pulmonary hypertension (n = 4) treated with inhaled nitric oxide (100% vs 31%; p = 0.01), sildenafil (100% vs 15%; p = 0.001), and bosentan (100% vs 6%; p < 0.001) during initial hospital stay was higher than that of patients without persistent pulmonary hypertension (n = 48). At 6 months, all patients with persistent pulmonary hypertension were tube-fed and treated with supplemental oxygen and sildenafil.

CONCLUSIONS:

Less than 10% of congenital diaphragmatic hernia patients had persistent pulmonary hypertension at ages 6 and/or 12 months. Follow-up for pulmonary hypertension should be reserved for congenital diaphragmatic hernia patients with echocardiographic signs of persistent pulmonary hypertension at hospital discharge and/or those treated with medication for pulmonary hypertension at hospital discharge.

PMID: 29419603

Curr Heart Fail Rep. 2018 Feb 7. doi: 10.1007/s11897-018-0377-9. [Epub ahead of print]

Pulmonary Vascular Disease: Hemodynamic Assessment and Treatment Selection-Focus on Group II Pulmonary Hypertension.

Ramu B¹, Houston BA¹, Tedford RJ².

Author information:

1. Division of Cardiology, Department of Medicine, Medical University of South Carolina (MUSC), Strom Thurmond Gazes Building, Room 215, 114 Doughty St/MSC592, Charleston, SC, 29425, USA.
2. Division of Cardiology, Department of Medicine, Medical University of South Carolina (MUSC), Strom Thurmond Gazes Building, Room 215, 114 Doughty St/MSC592, Charleston, SC, 29425, USA. TedfordR@musc.edu.

Abstract

PURPOSE OF REVIEW:

Pulmonary hypertension due to left heart disease (PH-LHD) is the most common cause of pulmonary hypertension worldwide, yet therapies used to treat pulmonary arterial hypertension have failed to show efficacy in this population. Proper hemodynamic assessment and differentiation of pulmonary hypertension phenotypes is therefore critical for both current clinical practice and future research and therapeutic efforts.

RECENT FINDINGS:

Substantial recent efforts have sought to improve the hemodynamic characterization of pulmonary hypertension for both diagnostic and prognostic purposes. These efforts include identifying occult LHD using provocative maneuvers as well as sub-classifying PH-LHD based on the presence or absence of a pre-capillary component. How to best define the pre-capillary component remains controversial as several studies have drawn conflicting conclusions. The lack of standardization of hemodynamic measurements as well as measurement fidelity concerns may explain some of the discrepant results. Non-hemodynamic methods of PH-LHD classification may also have an emerging role. Despite recent advances, therapeutic studies have largely remained disappointing. In this review, we discuss the nuances and controversies surrounding diagnostic and prognostic hemodynamic characterization of PH-LHD as well as summarize the recent therapeutic efforts and ongoing challenges in this population.

PMID: 29417467

Int J Chron Obstruct Pulmon Dis. 2018 Jan 26;13:385-397. doi: 10.2147/COPD.S152971. eCollection 2018.

Sex-specific cardiopulmonary exercise testing indices to estimate the severity of inoperable chronic thromboembolic pulmonary hypertension.

Chen TX¹, Pudasaini B¹, Guo J², Gong SG¹, Jiang R¹, Wang L¹, Zhao QH¹, Wu WH¹, Yuan P¹, Liu JM¹.

Author information:

1. Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University, Shanghai, People’s Republic of China.
2. Department of Pulmonary Function Test, Shanghai Pulmonary Hospital, Tongji University, Shanghai, People’s Republic of China.

Abstract

Background:

Sex differences in chronic thromboembolic pulmonary hypertension (CTEPH) have been revealed in few studies. Although right heart catheterization (RHC) is the gold standard for clinical diagnosis and assessment of prognosis in pulmonary hypertension (PH), cardiopulmonary exercise testing (CPET) has been a more widely used assessment of functional capacity, disease severity, prognosis, and treatment response in PH. We hypothesized that the “sex-specific” CPET indices could estimate the severity of inoperable CTEPH.

Methods:

Data were retrieved for 33 male (age, mean ± standard deviation [SD] =62.5±13.4 years) and 40 female (age, mean ± SD =56.3±11.8 years) patients with stable CTEPH who underwent both RHC and CPET at Shanghai Pulmonary Hospital from February 2010 to February 2016. Univariate and forward/backward multiple stepwise regression analysis was performed to assess the predictive value of CPET indices to hemodynamic parameters. Event-free survival was estimated using the Kaplan-Meier method and analyzed with the log-rank test. Cox proportional hazards models were performed to determine the independent event-free survival predictors.

Results:

Numerous CPET parameters were different between male and female patients with CTEPH and the control group. There were no significant differences in both clinical variables and RHC parameters between male and female patients with CTEPH. O2 pulse, workload, minute ventilation (VE), and end-tidal partial pressure of O2 (PETO2) at anaerobic threshold, as well as peak O2 pulse, workload, VE, and nadir VE/CO2 were significantly higher in male patients than in female patients (P<0.05). Only oxygen uptake efficiency plateau (OUEP) showed a significantly higher difference in female than male patients (P<0.05). In addition, several CPET indices correlated with hemodynamic parameters, especially pulmonary vascular resistance (PVR), which was distinctly different between the sexes. Nadir VE/CO2 was an independent predictor of PVR in male patients with CTEPH, whereas OUEP was an independent predictor of PVR in female patients with CTEPH.

Conclusion:

Even after confounding for age and body mass index, different CPET measurements of gas exchange efficiency correlated with PVR differently between male and female patients. This potentially could be used to estimate the severity of CTEPH.

PMCID: PMC5790096 Free PMC Article
PMID: 29416329

Respirology. 2018 Feb 7. doi: 10.1111/resp.13263. [Epub ahead of print]

Screening for pulmonary hypertension in interstitial lung disease: Many reasons to ECHO!

Prasad JD^1,2.

Author information:

1. Respiratory Department, Alfred Hospital, Melbourne, VIC, Australia.
2. Respiratory Department, Royal Melbourne Hospital, Melbourne, VIC, Australia.

PMID: 29415370

Interact Cardiovasc Thorac Surg. 2017 Dec 1;25(6):930-936. doi: 10.1093/icvts/ivx233.

Cardiopulmonary bypass does not induce lung dysfunction after pulmonary thrombarterectomy: role of pulmonary compliance.

Sacuto T¹, Sacuto Y².

Author information:

1. Department of Anesthesiology and Intensive Care, Marie Lannelongue Hospital, Le Plessis, Robinson, France.
2. Department of Anesthesiology and Intensive Care, Rouen University Hospital, Rouen, France.

Abstract

OBJECTIVES:

Pulmonary endarterectomy is a heavy surgical procedure that is performed under cardiopulmonary bypass (CPB) and aimed to cure postembolic pulmonary hypertension. Reperfusion oedema is both the hallmark of successful surgical procedure and the most frequent postoperative complication. Post-CPB lung dysfunction was not mentioned in any report. We undertook a study to determine whether post-CPB lung dysfunction was present in these patients.

METHODS:

In a retrospective cohort study with matching on some baseline covariates, we selected 41 patients who had undergone pulmonary endarterectomy and in whom pre-, intra- and postoperative records were complete. The control group was composed of 39 patients operated on from elective cardiac surgery during the same period and matched with a study group for age, gender, body mass index, blood creatinine, diabetes and baseline partial pressure of oxygen/fraction of inspired oxygen ratio. Criteria for post-CPB lung dysfunction were partial pressure of oxygen/fraction of inspired oxygen ratio decrease and bilateral basal oedema. Explanatory variables for post-CPB lung dysfunction were coronary arterial bypass, pleura opening, static pulmonary compliance measured at the time of thorax closed then retracted, fluid infusion, transfusion and vasopressors.

RESULTS:

All patients operated on from pulmonary endarterectomy presented radiological oedema reperfusion in surgical unblocking areas. Among them, only 2 had bilateral basal oedema when compared to the 24 patients from the control group (P < 0.001). Partial pressure of oxygen/fraction of inspired oxygen ratio increased in the study group and decreased in the control group (30 ± 109 vs -67 ± 134 mmHg, P < 0.001). Control group patients with high-baseline pulmonary compliance were at risk for post-CPB lung dysfunction.

CONCLUSIONS:

Patients operated on from pulmonary endarterectomy were saved from post-CPB lung dysfunction. The latter could be induced by a mechanical phenomenon.

PMID: 29049633 [Indexed for MEDLINE]

Int J Cardiol. 2017 Nov 1;246:59-60. doi: 10.1016/j.ijcard.2017.03.017.

Skeletal muscle exercise training in pulmonary arterial hypertension.

Madden BP¹, Shaw EJ².

Author information:

1. Department of Cardiothoracic Medicine, St Georges Hospital, Blackshaw Road, London SW17 0QT, United Kingdom. Electronic address: brendan.madden@stgeorges.nhs.uk.
2. Department of Cardiothoracic Medicine, St Georges Hospital, Blackshaw Road, London SW17 0QT, United Kingdom.

Comment on

Benefits of skeletal-muscle exercise training in pulmonary arterial hypertension: The WHOLEi+12 trial. [Int J Cardiol. 2017]

PMID: 28867020 [Indexed for MEDLINE]

Interact Cardiovasc Thorac Surg. 2017 Nov 1;25(5):740-744. doi: 10.1093/icvts/ivx158.

Aortopulmonary window: results of repair beyond infancy.

Talwar S¹, Siddharth B¹, Gupta SK¹, Choudhary SK¹, Kothari SS¹, Juneja R¹, Saxena A¹, Airan B¹.

Author information:

1. Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India.

Abstract

OBJECTIVES:

To study the anatomic and haemodynamic data and results of surgery in patients undergoing surgical repair of aortopulmonary window beyond infancy.

METHODS:

Between July 2005 and December 2015, 23 patients, older than 1 year undergoing surgery for aortopulmonary window were analysed retrospectively. Postoperative clinical and echocardiography follow-up were performed.

RESULTS:

Median age and weight at repair was 4 years (range 14 months-12 years) and 12 kg (range 3.5-22 kg), respectively. Fifteen patients had Richardson’s Type I, 6 patients had Type II and 2 patients had Type III aortopulmonary window. Six patients had associated defects. Baseline mean systolic pulmonary artery pressure was 101 ± 14.9 mmHg (range 80-130, median 100 mmHg) and pulmonary vascular resistance index was 9.6 ± 5.9 (median 7.7 Wood units/m2, range 3.7-23.5 Wood units/m2). Patch repair of aortopulmonary window was performed using the sandwich method (transwindow) (n = 15), transaortic (n = 3) and transpulmonary artery (n = 2) approaches; 2 patients underwent double ligation and 1 underwent division and suturing. Two patients underwent valved patch closure of aortopulmonary window and 1 patient underwent valved patch closure of associated ventricular septal defect. There were 2 in-hospital deaths: one due to intractable pulmonary hypertension and the other due to low cardiac output. Mean follow-up was 36 months (range 2-119 months). Eighteen patients were in NYHA Class I at last follow-up. There were no late deaths or reoperation.

CONCLUSIONS:

Surgery can be safely undertaken beyond infancy in carefully selected patients of aortopulmonary window with acceptable early and mid-term outcomes.

PMID: 28633352 [Indexed for MEDLINE]

J Clin Ultrasound. 2017 Jan;45(1):28-34. doi: 10.1002/jcu.22396. Epub 2016 Sep 13.

A comparison of echocardiographic variables of right ventricular function with exercise capacity after bosentan treatment in patients with pulmonary arterial hypertension: Results from a multicenter, prospective, cohort study.

Kim H¹, Bae Lee J², Park JH³, Yoo BS⁴, Son JW⁵, Yang DH⁶, Lee BR⁷.

Author information:

1. Division of Cardiology, Department of Internal Medicine, Keimyung University Dongsan Medical Center, Daegu, Korea.
2. Division of Cardiology, Department of Internal Medicine, Daegu Catholic University Medical Center, Daegu, Korea.
3. Cardiology Division in Internal Medicine, Chungnam National University Hospital, Daejeon, Korea.
4. Division of Cardiology, Department of Internal Medicine, Yonsei University Wonju Severance Christian Hospital, Wonju, Korea.
5. Division of Cardiology, Yeungnam University Medical Center, Yeungnam University College of Medicine, Daegu, Korea.
6. Division of Cardiology, Department of Internal Medicine, Kyungpook National University School of Medicine, Daegu, Korea.
7. Department of Cardiology, Fatima General Hospital, Daegu, Korea.

Abstract

PURPOSE:

Bosentan reduces pulmonary arterial pressure and improves exercise capacity in patients with pulmonary arterial hypertension (PAH). However, there are limited data regarding the extent to which the changes in echocardiographic variables reflect improvements in exercise capacity. We aimed to assess the improvement of echocardiographic variables and exercise capacity after 6 months of bosentan treatment for PAH.

METHODS:

We performed a prospective study from June 2012 to June 2015 in seven participating medical centers. Echocardiography, including tissue Doppler imaging (TDI) and the 6-minute walk test distance (6MWD), was performed at baseline and after 6 months of bosentan treatment.

RESULTS:

We analyzed 19 patients with PAH: seven with congenital shunt, six with collagen vascular disease, and six with idiopathic PAH. After bosentan treatment, mean 6MWD increased by 50 meters. Right ventricle (RV) systolic pressure, tricuspid annular plane systolic excursion, myocardial performance index (MPI) derived from TDI (MPI-TDI) of RV and left ventricle (LV), RV fractional area change, and RV ejection fraction were significantly improved. In particular, the magnitude of RV and LV MPI-TDI showed good correlation with changes in the 6MWD.

CONCLUSIONS:

The magnitude of RV and LV MPI-TDI was strongly associated with improvements in exercise capacity. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:28-34, 2017.

© 2016 Wiley Periodicals, Inc.

PMID: 27619758 [Indexed for MEDLINE]

J Cardiothorac Vasc Anesth. 2017 Jun 16. pii: S1053-0770(17)30570-0. doi: 10.1053/j.jvca.2017.06.028. [Epub ahead of print]

Systolic Anterior Motion of the Mitral Valve During Pulmonary Endarterectomy in a Patient with Chronic Thromboembolic Pulmonary Hypertension: A Case Report.

Hoshino K¹, Takita K², Demura M², Kubo T², Morimoto Y².

Author information:

1. Department of Anesthesiology and Critical Care Medicine, Hokkaido University Hospital, Sapporo, Japan. Electronic address: hoshinoko@med.hokudai.ac.jp.
2. Department of Anesthesiology and Critical Care Medicine, Hokkaido University Hospital, Sapporo, Japan.

PMID: 29398378