Cardiac interventions in patients with achondroplasia: a systematic review.

Patients with achondroplasia and other causes of dwarfism suffer from increased rates of cardiovascular disease relative to the remainder of the population. Few studies have examined these patients when undergoing cardiac surgery or percutaneous intervention. This systematic review examines the literature to determine outcomes following cardiac intervention in this unique population. An electronic search was performed in the English literature to identify all reports of achondroplasia, dwarfism, and cardiac intervention. Of the 5,274 articles identified, 14 articles with 14 cases met inclusion criteria. Patient-level data was extracted and analyzed. Median patient age was 55.5 [interquartile ranges (IQR), 43.8, 59.8] years, median height 102.0 [98.8, 112.5] cm, median BMI 32.1 [27.0, 45.9], and 57.1% (8/14) were male. Of these 14 patients, nine had the following documented skeletal abnormalities: 66.7% (6/9) had scoliosis, 66.7% (6/9) had kyphosis, 11.1% (1/9) had lordosis, 11.1% (1/9) pectus carinatum and 11.1% (1/9) spinal stenosis. Coronary artery disease was present in 53.8% (7/13), and 30.8% (4/13) patients previously suffered a myocardial infarction. Of the eight patients who underwent cardiac surgery, 37.5% (3/8) underwent multivessel coronary artery bypass grafting, 37.5% (3/8) underwent aortic valve replacement, 25.0% (2/8) underwent type A aortic dissection repair, and the remaining 12.5% (1/8) underwent pulmonary thromboendarterectomy. Six patients underwent percutaneous intervention. Median cardiopulmonary bypass time was 136.5 [110.0, 178.8] minutes. Median arterial cannula size was 20.0 [20.0, 24.0] Fr. Bicaval cannulation was performed in all cases describing cannulation strategy (5/5). Median superior vena cava cannula size was 28.0 [28.0, 28.0] Fr, and inferior vena cava cannula size was 28.0 [28.0, 28.0] Fr. No mortality was reported with a median follow up time of 6.0 [6.0, 10.5] months. In conclusion, Common cardiac procedures can be performed with reasonable safety in this patient population. Operative adjustments may need to be made with respect to equipment to accommodate patient-specific needs

Continuous-flow left ventricular assist device outflow graft stenting: Indications and outcomes

Introduction: Stenosis in the continuous-flow left ventricular assist device (CF-LVAD) outflow graft can be caused by various mechanical and anatomical factors. Increasingly, percutaneous management has been utilized to re-establish adequate CF-LVAD flow. We sought to evaluate indications for such interventions and their outcomes. Methods: An electronic search was performed to identify all studies in the English literature reporting CF-LVAD outflow graft stenting for various etiologies. Twenty-one studies consisting of 26 patients were included in the analysis. Results: Median patient age was 59 years [45.8-67.0] and 65.4% (17/26) were male. 58.3% (14/24) of patients had HeartWare HVAD, 37.5% (9/24) had HeartMate II LVAD, and 4.2% (1/24) had HeartMate III LVAS. Median time from device placement to outflow graft stenting was 24.0 months [7.8-30.4]. 76.9% of patients (20/26) presented with heart failure. 34.6% (9/26) had outflow graft thrombosis, 34.6% (9/26) stenosis, 11.5% (3/26) kinking, 11.5% (3/26) pseudoaneurysm, 3.8% (1/26) external graft compression, and 3.8% (1/26) had a bronchialarterial fistula. 88.5% (23/26) procedures led to immediate flow improvement with the remaining 11.5% (3/26) receiving additional stenting. Post-intervention flows were significantly improved (4.7 L/min [4.1-4.8] post-intervention vs 2.9 L/min [2.0-3.5] initial, p=0.01). 96.2% (25/26) patients were discharged from the hospital. The 30-day mortality was 6.7% (1/15). Overall mortality during the median follow-up of 90 days [7.0-240.0] was 9.5% (2/21). Discussion: Outflow graft stenting appears to effectively alleviate CF-LVAD outflow graft obstruction with low mortality. Longer-term follow up is necessary to determine the longevity of such an intervention but early results are promising

Does Concomitant CABG Influence the Outcomes of Post-Myocardial Infarction Ventricular Septal Defect Repair?

Introduction: Ventricular septal defect (VSD) following myocardial infarction (MI) is a relatively infrequent complication with high mortality. Over time, understanding of the pathology and its management has resulted in improved outcomes; however, controversies remain. Objective: We sought to investigate the effect of concomitant coronary artery bypass graft (CABG) on outcomes following post-MI VSD repair. Methods: Electronic search was performed to identify all relevant studies published from 2000 to 2018. After assessment for inclusion and exclusion criteria, 66 studies were selected for the analysis. Data were extracted and pooled for systematic review and meta-analysis. Results: Average age was 68.7 years (95% CI 67.3-70.1) with 57% (95% CI 54-60) males. Coronary angiogram was available preoperatively in 94% (95% CI 92-96) of patients. Single-vessel disease was most common (47%, 95% CI 42-52) with left anterior descending coronary artery the most commonly involved vessel (55%, 95% CI 46-63). Concomitant CABG was performed in 52% (95% CI 46-57) of patients. Of these, infarcted territory was revascularized in 54% (95% CI 23-82). No significant survival difference was observed between those who had concomitant CABG versus those without CABG at 30 days (65%, 95% CI 58-72) vs (60%, 95% CI 47-72), 1 year (59%, 95% CI 50-68) vs (51%, 95% CI 41-61), and 5 years (46%, 95% CI 38-54) vs (39%, 95% CI 27-52) respectively. Discussion: Overall, concomitant CABG did not have a significant effect on survival following VSD repair, therefore, decision on revascularization should be weighed against the risks associated with prolonged cardiopulmonary bypass

Bronchial artery revascularization in lung transplantation: a systematic review and meta-analysis.

Background: Bronchial artery revascularization (BAR) during lung transplantation has been hypothesized to improve early tracheal healing and delay the onset of bronchiolitis obliterans syndrome (BOS). We aimed to assess the outcomes of BAR after lung transplantation. Methods: Electronic search in Ovid Medline, Cumulative Index of Nursing and Allied Health Literature (CINAHL), Scopus, and Cochrane Controlled Trials Register (CCTR) databases was performed to identify all relevant studies published about lung transplantation with BAR. Studies discussing lung transplantation utilizing BAR were included while those without outcome data such as BOS and survival were excluded. Cohort-level data were extracted and pooled for analysis. A binary outcome meta-analysis of proportions with logit transformation was conducted. Newcastle-Ottawa scale was used for risk of bias assessment. Results: Seven studies were selected for the analysis comprising 143 patients. Mean patient age was 47 (95% CI: 40-55) years. Sixty-one percent (48-72%) were male. Seventy-three percent (65-79%) of patients underwent double lung transplant while 27% (21-25%) underwent single lung transplant. In patients with postoperative angiography, successful BAR was demonstrated in 93% (82-97%) of all assessed conduits. The 30-day/in-hospital mortality was 6% (3-11%). Seventy-nine percent (63-89%) of patients were free from rejection at three months. Eighty-three percent (29-98%) of patients were free from signs of airway ischemia at three and six months. Pooled survival at one year and five years was 87% (78-92%) and 71% (46-87%), respectively, with a mean follow-up time of 21 (3-38) months. Pooled freedom from bronchiolitis obliterans was 86% (77-91%) at two years. Conclusions: While this systematic review and meta-analysis is limited by the available surgeons, institutions, and papers discussing a highly specialized technique, it does show that BAR is a viable technique to minimize BOS and early anastomotic intervention following lung transplantation

Surgical Pulmonary Embolectomy Outcomes for Acute Pulmonary Embolism

Introduction: Acute pulmonary embolism (PE) is associated with significant mortality. Surgical embolectomy is a viable treatment option; however, it remains controversial due to variable outcomes. This review investigates patient outcomes following surgical embolectomy for acute PE. Methods: Electronic search was performed to identify articles reporting surgical embolectomy for treatment of PE. 32 studies were included comprising 936 patients. Demographic, perioperative, and outcome data were extracted and pooled for systematic review. Results: Mean patient age was 56.3 [95% CI 52.5; 60.1] years and 50% [46; 55] were male. 82% had right ventricular dysfunction [62; 93], 80% [67; 89] had unstable hemodynamics, and 9% [5; 16] experienced cardiac arrest. Massive PE and submassive PE were present in 83% of patients [43; 97] and 13% [2; 56], respectively. Before embolectomy, 33% of patients [14; 60] underwent systemic thrombolysis and 14% [8; 24] catheter embolectomy. Preoperatively, 47% of patients were ventilated [26; 70] and 36% had percutaneous cardiopulmonary support [11; 71]. Mean operative time and mean cardiopulmonary bypass time were 170 [101; 239] and 56 [42; 70] minutes, respectively. Intraoperative mortality was 4% [2; 8]. Mean hospital and ICU stay were 10 [6; 14] and 2 [1; 3] days, respectively. Mean postoperative systolic pulmonary artery pressure (sPAP) was significantly decreased from preoperative (sPAP 57.8 mmHg [53; 62.7]) to postoperative period (sPAP 31.3 mmHg [24.9; 37.8]), p \u3c0.01). In-hospital mortality was 16% [12; 21]. Overall survival at five years was 73% [64; 81]. Discussion: Surgical embolectomy is an acceptable treatment option with favorable outcomes

Endovascular Intervention for Tracheo-Innominate Fistula: A Systematic Review and Meta-analysis

Introduction: Fistula formation between the trachea and the innominate artery is a life-threatening complication rarely seen with existing or previous tracheostomy. Fatal upon rupture, swift diagnosis and immediate intervention are paramount for survival. We aim to identify feasibility and outcomes of endovascular intervention for trachea-innominate fistula (TIF). Methods: Patient-level data of reported individuals above the age of 14 that underwent endovascular intervention for TIF was extracted and analyzed. Identification of 25 patients from 27 studies was accomplished by electronic database search of Cochrane Central Register of Controlled Trials, Cumulative Index to Nursing and Allied Health Literature, Ovid Medline, and Scopus. Survival data was evaluated by Kaplan-Meier analysis. Results: Median patient age was 39.0 years [IQR 16.0, 47.5]. Median time to TIF presentation following tracheostomy was 2.2 months [0.5, 42.5]. 84.6% (22/27) exhibited tracheal hemorrhage at presentation. Covered stent graft placement was performed in 96.3% (26/27) and coil embolization in 3.8% (1/27). Repeat endovascular intervention was necessary in 18.5% (5/27) and rescue sternotomy was required in 11.1% (3/27). Overall mortality was 29.6% (8/27) with a median follow-up time of 5 months [1.2, 11.5]. Discussion: Endovascular intervention may be an effective method of TIF repair at presentation. As an alternative to conventional surgical repair, endovascular intervention may be an appropriate method for TIF repair particularly in patients unfit for open sternotomy repair

Development of malignancies and their outcomes in patients supported on continuous-flow left ventricular assist devices-a systematic review.

Background: With increased use of continuous-flow left ventricular assist devices (CF-LVAD), development of malignant tumors in this population is not uncommon. We sought to evaluate malignancies in CF-LVAD patients and evaluate the outcomes of treatment strategies. Methods: Overall, 18 articles consisting of 28 patients were identified who developed malignancies after CF-LVAD placement. Patient-level data were extracted for systematic review. Results: Median patient age was 60 years [59-67] and 85.7% (24/28) were male. CF-LVAD was placed as bridge-to-transplant (BTT) in 60.9% (14/23) of patients. The three most common malignancy types were GI in 35.7% (10/28) of patients, lung in 21.4% (6/28) and skin in 10.7% (3/28). Median time from CF-LVAD implant to malignancy diagnosis was 6.9 [2.5-12.8] months. Metastatic disease occurred in 17.9% (5/28) over a median time of 5.0 [1.0-82.0] months from the diagnosis. Surgical resection of the malignancy was performed in 57.1% (16/28) of patients. Our results showed that while there was a significantly higher probability of survival among patients who underwent surgery versus those who did not, when only stage I and II patients were included in the analysis, this difference was no longer statistically significant. Three patients were relisted for heart transplant after surgical treatment, and two received the transplant. Conclusions: Surgical management of malignancies in patients on CF-LVADs may improve survival and transplant eligibility status, therefore, a CF-LVAD should not always preclude surgical treatment

Outcomes of extracorporeal life support for the treatment of acute massive pulmonary embolism: a systematic review

Background: Massive pulmonary embolism (PE) can cause hemodynamic instability leading to high mortality. Extracorporeal life support (ECLS) has been increasingly used as a bridge to definitive therapy. This systematic review investigates the outcomes of ECLS for the treatment of massive PE. Methods: Electronic search was performed to identify all relevant studies published on ECLS use in patients with PE. 50 case series or reports were selected comprising 128 patients with acute massive PE who required ECLS. Patient-level data were extracted for statistical analysis. Results: Median patient age was 50 [36, 63] years and 41.3% (50/121) were male. 67.2% (86/128) of patients presented with cardiac arrest. Median heart rate was 126 [118, 135] and median systolic pulmonary artery pressure (sPAP) was 55 [48, 69] mmHg. The majority of ECLS included veno-arterial ECLS [97.1% (99/102)]. Median ECLS time was 3 [2, 6] days. 43.0 0 0 (55/128) patients received systemic thrombolysis, 22.7% (29/128), received catheter-guided thrombolysis, and 37.5% (48/128) underwent surgical embolectomy. 85.1% (97/114) were weaned off ECLS. Post-ECLS complications included bleeding in 23.4% (30/128), acute renal failure in 8.6% (11/128), dialysis in 6.3% (8/128), heparin-induced thrombocytopenia in 3.1 (4/128), and extremity hypoperfusion in 2.3% (3/128). The most common cause of death was shock at 30.3% (10/33). The median length of hospital stay was 22 [11, 39] days including 8 [5, 13] intensive care unit (ICU) days. The 30-day mortality rate was 22% (20/91). Conclusions: ECLS is safe and effective therapy in unstable patients with acute massive pulmonary embolism and offers acceptable outcomes

Outcomes of Mechanical Circulatory Support for Giant Cell Myocarditis: A Systematic Review

Treatment of giant cell myocarditis (GCM) can require bridging to orthotopic heart transplantation (OHT) or recovery with mechanical circulatory support (MCS). Since the roles of MCS and immunotherapy are not well-defined in GCM, we sought to analyze outcomes of patients with GCM who required MCS. A systematic search was performed in June 2019 to identify all studies of biopsy-proven GCM requiring MCS after 2009. We identified 27 studies with 43 patients. Patient-level data were extracted for analysis. Median patient age was 45 (interquartile range (IQR): 32–57) years. 42.1% (16/38) were female. 34.9% (15/43) presented in acute heart failure. 20.9% (9/43) presented in cardiogenic shock. Biventricular (BiVAD) MCS was required in 76.7% (33/43) of cases. Of the 62.8% (27/43) of patients who received immunotherapy, 81.5% (22/27) used steroids combined with at least one other immunosuppressant. Cyclosporine was the most common non-steroidal agent, used in 40.7% (11/27) of regimens. Immunosuppression was initiated before MCS in 59.3% (16/27) of cases, after MCS in 29.6% (8/27), and not specified in 11.1% (3/27). Immunosuppression started prior to MCS was associated with significantly better survival than MCS alone (p = 0.006); 60.5% (26/43) of patients received bridge-to-transplant MCS; 39.5% (17/43) received bridge-to-recovery MCS; 58.5% (24/41) underwent OHT a median of 104 (58–255) days from diagnosis. GCM recurrence after OHT was reported in 8.3% (2/24) of transplanted cases. BiVAD predominates in mechanically supported patients with GCM. Survival and bridge to recovery appear better in patients on immunosuppression, especially if initiated before MCS