Long-term outcomes in heritable thoracic aortic disease

Heritable aortic aneurysm is an increasingly recognized cause of morbidity and mortality. Whilst Marfan syndrome (MFS) is well-known, the clinical presentation and prognosis of more newly described genetic syndromes is less familiar to clinicians. There is a particular lack of knowledge regarding clinical outcomes for non-syndromal heritable aortic disease. This study investigated the presentation, clinical course and survival of patients with syndromal [Loeys-Dietz, aneurysm-osteoarthritis, and aneurysm-cerebral arteriopathy (ACTA2) syndrome] and non-syndromal heritable aortic disease in comparison to MFS. The study group includes 536 individuals (283 Marfan, 176 non-syndromal heritable aortopathy, 36 aneurysm-osteoarthritis, 32 Loeys-Dietz, and 9 ACTA2 aneurysm) enrolled in a longitudinal clinical follow-up between 1990 and 2022. Age at diagnosis differed between groups: Marfan = 22.0 ± 16.6; Loeys-Dietz = 29.6 ± 21.5; aneurysm-osteoarthritis = 36.4 ± 18.8; ACTA2 aneurysm = 43.4 ± 18.6; non-syndromal heritable aortopathy = 47.2 ± 16.6 years (p < 0.001). Aortic dissection was the presenting event in 8% individuals with Marfan compared to 27% with non-syndromal heritable aortopathy and 34% with Loeys-Dietz syndrome (p < 0.01). Mean follow-up duration for the group was 16.4 years (range 0.2-30 years) and 74 individuals died during follow-up (Marfan = 52, Loeys-Dietz = 6, aneurysm-osteoarthritis = 4, ACTA2 aneurysm = 1, heritable non-syndromal aortopathy = 11). At 10 years follow-up, actuarial mean survivals were: aneurysm-osteoarthritis = 77.5 ± 10.4%; Loeys-Dietz = 90.0 ± 6.8%; Marfan = 94.6 ± 1.4%; heritable non-syndromal aortopathy = 95.9 ± 2.1% (NS). There were 60 aortic dissections (24 Type A, 36 Type B) during follow-up. At 10 years, survival free of dissection was comparable between groups: aneurysm-osteoarthritis = 90.7 ± 6.4%; Loeys-Dietz = 94.4 ± 5.4%; Marfan = 96.1 ± 1.2%; heritable non-syndromal aortopathy = 93.9 ± 2.3%, with similar findings at 20 years. Prophylactic aortic surgery was a first event during follow-up for 196 individuals (ACTA2 aneurysm = 3; aneurysm-osteoarthritis = 10; Loeys-Dietz = 19; Marfan = 119; heritable non-syndromal aortopathy = 45). A second surgical intervention was required in 45 individuals and a third intervention in 21 individuals. At 10 years follow-up, survival free of surgery differed between groups: aneurysm-osteoarthritis = 68.5 ± 10.1%; Loeys-Dietz = 40.8 ± 11.2%; Marfan = 75.5 ± 2.7%; heritable non-syndromal aortopathy = 63.8 ± 4.7% (p < 0.001). At 20 years follow-up mean survival free of surgery was: aneurysm-osteoarthritis = 26.6 ± 14.7%; Loeys-Dietz = 9.1 ± 8.2%; Marfan = 57.2 ± 3.4%; heritable non-syndromal aortopathy = 41.6 ± 8.2% (p < 0.001). Diagnosis of newer syndromic and non-syndromal heritable aortopathies is delayed compared to MFS, with associated complications of presentation with aortic dissection. Survival of individuals enrolled in follow-up surveillance is comparable between different genetic aortopathies, however aortic dissections still occur and need for surgical intervention is high.

Ventricular Septal Myectomy for Obstructive Hypertrophic Cardiomyopathy (Analysis Spanning 60 Years Of Practice): AJC Expert Panel.

Surgical myectomy remains the time-honored primary treatment for hypertrophic cardiomyopathy patients with drug refractory limiting symptoms due to LV outflow obstruction. Based on >50 years experience, surgery reliably reverses disabling heart failure by permanently abolishing mechanical outflow impedance and mitral regurgitation, with normalization of LV pressures and preserved systolic function. A consortium of 10 international currently active myectomy centers report about 11,000 operations, increasing significantly in number over the most recent 15 years. Performed in experienced multidisciplinary institutions, perioperative mortality for myectomy has declined to 0.6%, becoming one of the safest currently performed open-heart procedures. Extended myectomy relieves symptoms in >90% of patients by ≥ 1 NYHA functional class, returning most to normal daily activity, and also with a long-term survival benefit; concomitant Cox-Maze procedure can reduce the number of atrial fibrillation episodes. Surgery, preferably performed in high volume clinical environments, continues to flourish as a guideline-based and preferred high benefit: low treatment risk option for adults and children with drug refractory disabling symptoms from obstruction, despite prior challenges: higher operative mortality/skepticism in 1960s/1970s; dual-chamber pacing in 1990s, alcohol ablation in 2000s, and now introduction of novel negative inotropic drugs potentially useful for symptom management.

Aortic root replacement in bicuspid versus tricuspid aortic valve patients.

Background: Concomitant replacement of the aortic root and aortic valve is a widely used treatment strategy in elective patients with aortic valve stenosis and root aneurysm. It is also a strategy frequently employed in patients with acute aortic dissection type A (AADA), involving the aortic root. Although more patients have undergone valve sparing procedures over the past decades, the classic ‘modified Bentall technique’ remains a valid option, particularly for patients with a bicuspid aortic valve (BAV). We aimed to compare the results of elective and emergency modified Bentall procedures in patients with bicuspid and tricuspid aortic valves (TAVs).

Methods: We retrospectively reviewed our database for patients undergoing either elective or emergency modified Bentall procedures between 2000 and 2018 and identified 827 elective cases (44% BAV) and 258 emergency cases (15% BAV). Analysis of intra- and postoperative outcomes and early mortality was performed. Due to inequality of the groups, a matching analysis was performed.

Results: We found BAV patients to be significantly younger (elective: 58±18 vs. 65±14, P<0.001; emergency: 49±17 vs. 62±19, P<0.001) and healthier at time of surgery. In the AADA cohort, malperfusion rate was not different between bicuspid and tricuspid patients, however bicuspid AADA patients presented more often with an entry in the aortic root. After matching, procedure times and early outcomes did not differ between the groups, except for significantly higher rates of respiratory failure in elective TAV patients (10% vs. 5%, P=0.033). The 30-day mortality was 2% in elective cases and 22% in emergency AADA surgery. A subgroup analysis of elective patients with aortic diameter <55 mm also showed excellent outcomes.

Conclusions: After adjustment for preoperative inequalities, no differences in early mortality and outcomes were found between bicuspid and tricuspid patients receiving elective or emergency modified Bentall surgery.

Complications of Robotic Video-Assisted Thoracoscopic Surgery Compared to Open Thoracotomy for Resectable Non-Small Cell Lung Cancer

Abstract

(1) Background: Conventional open thoracotomy has been the accepted surgical treatment for resectable non-small cell lung cancer. However, newer, minimally invasive approaches, such as robotic surgery, have demonstrated similar safety and efficacy with potentially superior peri-operative outcomes. The present study aimed to quantitatively assess these outcomes through a meta-analysis. (2) Methods: A systematic review was performed using electronic databases to identify all of the relevant studies that compared robotic surgery with open thoracotomy for non-small cell lung cancer. Pooled data on the peri-operative outcomes were then meta-analyzed. (3) Results: Twenty-two studies involving 12,061 patients who underwent robotic lung resection and 92,411 patients who underwent open thoracotomy were included for analysis. Mortality rates and length of hospital stay were significantly lower in patients who underwent robotic resection. Compared to open thoracotomy, robotic surgery was also associated with significantly lower rates of overall complications, including atrial arrhythmia, post-operative blood transfusions, pneumonia and atelectasis. However, the operative times were significantly longer with robotic lung resection. (4) Conclusions: The present meta-analysis demonstrated superior post-operative morbidity and mortality outcomes with robotic lung resection compared to open thoracotomy for non-small cell lung cancer.

Virulence of Staphylococcus Infection in Surgically Treated Patients With Endocarditis : A Multicenter Analysis.

Objective: Infective endocarditis (IE) caused by Staphylococcus species (spp.) is believed to be associated with higher morbidity and mortality rates. We hypothesize that Staphylococcus spp. are more virulent compared with other commonly causative bacteria of IE with regard to short-term and long-term mortality.

Background: It remains unclear if patients suffering from IE due to Staphylococcus spp. should be referred for surgical treatment earlier than other IE patients to avoid septic embolism and to optimize perioperative outcomes.

Materials and methods: The database of the CAMPAIGN registry, comprising 4917 consecutive patients undergoing heart valve surgery, was retrospectively analyzed. Patients were divided into 2 groups with regard to the identified microorganisms: Staphylococcus group and the non- Staphylococcus group. The non- Staphylococcus group was subdivided for further analyses: Streptococcus group, Enterococcus group, and all other bacteria groups.

Results: The respective mortality rates at 30 days (18.7% vs 11.8%; P <0.001), 1 year (24.7% vs 17.7%; P <0.001), and 5 years (32.2% vs 24.5%; P <0.001) were significantly higher in Staphylococcus patients (n=1260) compared with the non- Staphylococcus group (n=1787). Multivariate regression identified left ventricular ejection fraction <30% ( P <0.001), chronic obstructive pulmonary disease ( P =0.045), renal insufficiency ( P =0.002), Staphylococcus spp. ( P =0.032), and Streptococcus spp. ( P =0.013) as independent risk factors for 30-day mortality. Independent risk factors for 1-year mortality were identified as: age ( P <0.001), female sex ( P =0.018), diabetes ( P =0.018), preoperative stroke ( P =0.039), chronic obstructive pulmonary disease ( P =0.001), preoperative dialysis ( P <0.001), and valve vegetations ( P =0.004).

Conclusions: Staphylococcus endocarditis is associated with an almost twice as high 30-day mortality and significantly inferior long-term outcome compared with IE by other commonly causative bacteria. Patients with Staphylococcus infection are more often female and critically ill, with >50% of these patients suffering from clinically relevant septic embolism. Early diagnosis and referral to a specialized center for surgical treatment are strongly recommended to reduce the incidence of preoperative deterioration and stroke due to septic embolism.

Clinical event rate in patients with and without left main disease undergoing isolated CABG: results from the European DuraGraft registry

Objectives: Left main coronary artery disease (LMCAD) is considered an independent risk factor for clinical events after coronary artery bypass grafting (CABG). We have conducted a subgroup analysis of the multicentre European DuraGraft registry to investigate clinical event-rates at 1-year in patients with and without LMCAD undergoing isolated CABG in contemporary practice.

Methods: Patients undergoing isolated CABG were selected. The primary end-point was the incidence of a major adverse cardiac event (MACE) defined as the composite of death, myocardial infarction (MI) or repeat revascularization (RR) at 1-year. The secondary end-point was major adverse cardiac and cerebrovascular events (MACCE) defined as MACE plus stroke. Propensity score matching (PSM) was performed to balance for differences in baseline characteristics.

Results: LMCAD was present in 1,033 (41.2%) and absent in 1,477 (58.8%) patients. At 1-year, the MACE rate was higher for LMCAD patients (8.2% vs 5.1%, p = 0.002) driven by higher rates of death (5.4% vs 3.4%, p = 0.016), MI (3.0% vs 1.3%, p = 0.002) and numerically higher rates of RR (2.8% vs 1.8%, p = 0.13). The incidence of MACCE was 8.8% vs 6.6%, p = 0.043 with a stroke rate of 1.0% and 2.4%, p = 0.011, for LMCAD and non-LMCAD group, respectively. After PSM, the MACE rate was 8.0% vs 5.2%, p = 0.015. The incidence of death was 5.1% vs 3.7%, p = 0.10, MI 3.0% vs 1.4%, p = 0.020, and RR was 2.7% vs 1.6%, p = 0.090, for the LMCAD and non-LMCAD group, respectively. Less strokes occurred in LMCAD patients (1.0% vs 2.4%, p = 0.017). The MACCE rate was not different: 8.5% vs 6.7%, p = 0.12.

Conclusions: In this large registry, LMCAD was demonstrated to be an independent risk factor for MACE after isolated CABG. Conversely, the risk of stroke was lower in LMCAD patients.

Trial registration: ClinicalTrials.gov NCT02922088.

Stay in the loop

Subscribe to our Heart to Heart Newsletter to keep up with the latest developments in heart and lung research from The Baird Institute.

Honour a Loved One

  • Fundraise in memory of someone special to you.

Challenge Yourself

  • Run a marathon
  • Do a long bike ride
  • Walk 10km each day for a month
  • Do 50 sit ups every day for a week
  • Join an organised event such as the City to Surf

Organise a community event

  • Have a backyard sausage sizzle
  • Host a trivia night

Seek sponsorship to help you quit those bad habits

  • Give up smoking
  • Refrain from alcohol for a month or more

Celebrate Through Giving

  • Choose to give on your birthday: Instead of giving you gifts, ask your friends and family to donate to The Baird Institute.
  • Say “I do” to improving the lives of heart and lung patients: Invite guests to donate to The Baird Institute on your wedding day
  • Turn anniversaries or personal milestones into fundraising events.

Create a CrowdRaiser on GiveNow

  1. Go to CrowdRaiser for The Baird Institute.
  2. Click on the button “Fundraise for this cause” – just under the header image.
  3. Create your Crowdraiser. Fill in the requested details.
  4. Customise your campaign. Add images and messages to make your CrowdRaiser unique.
  5. Share the link to your fundraising page via email, social media, or any way you like.
  6. Let us know via [email protected] that you have created a fundraiser so we can say thank you.

Join a community passionate about making a difference. GiveNow provides a dedicated platform for Australian charities, ensuring your efforts directly support our mission.

Start a Facebook Fundraiser

  1. Go to Facebook fundraisers.
  2. Click on the blue button – “Select nonprofit”
  3. Search for and select The Baird Institute
  4. Set your fundraising target
  5. Choose your campaign end date & a title for your Fundraiser
  6. Personalise your fundraiser: Use the existing wording and photos or choose your own.
  7. Click on ‘Create’.
  8. Invite friends and family. Share the link for your fundraiser and encourage others to contribute.
  9. Let us know via [email protected] that you have created a fundraiser so we can say thank you.

Celebrate where your friends and family connect. Leverage your social network to make a real impact.