Acute Type A Dissection Repair in an Achondroplastic Dwarf: Anesthetic, Perfusion, and Surgical Concerns

Al-Jughiman, Mohammed; Yanagawa, Bobby; Rondi, Kevin; Dalamagas, Constantine; Peterson, Mark D.; Bonneau, D.

doi:10.12945/j.aorta.2014.14-020

Cited by 7 publications

(8 citation statements)

References 6 publications

(11 reference statements)

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“…Following intubation, intravascular access and patient positioning are both challenging due to short limbs, reduced joint mobility, and anatomy of the chest (8). Several reports note the use of ultrasound guidance for intravascular access and arterial and vein cut-downs for safe vascular exposure (10,11). These differences from the general population must be considered in preparing for procedures on patients with achondroplasia.…”

Section: Discussionmentioning

confidence: 99%

“…For procedures requiring artery or vein conduit, it should be noted that saphenous vein graft may be challenging to obtain, too short for purposes of a bypass, or unsuitable for bypass. One such report noted smaller length of saphenous vein harvest with moderate quality due to patient anatomy and performed a three-vessel coronary-artery bypass with both right and left internal mammary arteries (10). Another report noted a 29 cm saphenectomy incision that extended to the patient's ankle (12), and a third noted pre-operative phlebography to assess for vein quality (13).…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Cardiac interventions in patients with achondroplasia: a systematic review

Sukhavasi¹,

O’Malley²,

Maynes³

et al. 2020

J Thorac Dis

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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. Patientlevel 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. Nomortality 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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Cardiac interventions in patients with achondroplasia: a systematic review

Sukhavasi¹,

O’Malley²,

Maynes³

et al. 2020

J Thorac Dis

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“…Scafuri et al used a pediatric cannula for aortic valve replacement with root enlargement in a 56-year-old woman with achondroplasia; however, they encountered no problem during CPB [ 6 ]. Other studies have reported using adult cannulas for the repair of type A aortic dissection [ 7 ], aortic valve replacement [ 8 ], and pulmonary endarterectomy [ 9 ]. Despite no consensus on this matter, an adult cannulation can often be performed successfully and should be considered given that these studies reportedly mentioned increasing their CPB flow to maintain adequate tissue perfusion.…”

Section: Discussionmentioning

confidence: 99%

Acute type A aortic dissection repair in an octogenarian with achondroplasia: a case report

2018

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BackgroundAchondroplasia is an inherited disorder and the most common type of short-limbed dwarfism in human beings, affecting more than 250,000 individuals worldwide. To the best of our knowledge, no study has reported a correlation between achondroplasia and aortic dissection. Here, we report a rare case of acute type A aortic dissection repair in a patient with achondroplasia.Case presentationAn 82-year-old Japanese female with achondroplasia was admitted to our hospital because of acute-onset severe chest pain migration to her back accompanied by numbness and pain in the right lower limb. A computed tomography scan revealed acute type A aortic dissection with right leg ischemia because of an occlusion of the right common iliac artery. We successfully performed hemiarch repair.ConclusionsThis report presents the first case of a patient at such an advanced age with dwarfism and cardiac surgery and the second case to illustrate successful acute aortic dissection repair in achondroplasia. Of note, all procedures were performed without specialized equipment. Overall, this report adds to the experience of successful cardiac surgery in this unique patient population.

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“…In the present case, although the patient did not receive CSF drainage, he still fully recovered. Al-Jughiman et al [ 13 ] previously reported 1 male case of achondroplastic dwarfism who presented with acute type-A aortic dissection with aortic insufficiency. He underwent Bentall and hemiarch repairing surgery.…”

Section: Discussionmentioning

confidence: 99%

Application of thoracic endovascular aortic repair (TEVAR) in treating dwarfism with Stanford B aortic dissection

Qiu

Cai²,

Shu³

et al. 2018

Medicine

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Rationale:To apply thoracic endovascular aortic repair (TEVAR) to treat dwarfism complicated with Stanford B aortic dissection.Patient concerns:In this report, we presented a 63-year-old male patient of dwarfism complicated with Stanford B aortic dissection successfully treated with TEVAR.Diagnoses:He was diagnosed with dwarfism complicated with Stanford B aortic dissection.Interventions:After conservative treatment, the male patient underwent TEVAR at 1 week after hospitalization. After operation, he presented with numbness and weakness of his bilateral lower extremities, and these symptoms were significantly mitigated after effective treatment. At 1- and 3-week after TEVAR, the aorta status was maintained stable and restored.Outcomes:The patient obtained favorable clinical prognosis and was smoothly discharged. During subsequent follow-up, he remained physically stable.Lessons:TEVAR is probably an option for treating dwarfism complicated with Stanford B aortic dissection, which remains to be validated by subsequent studies with larger sample size.

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Acute Type A Dissection Repair in an Achondroplastic Dwarf: Anesthetic, Perfusion, and Surgical Concerns

Cited by 7 publications

References 6 publications

Cardiac interventions in patients with achondroplasia: a systematic review

Cardiac interventions in patients with achondroplasia: a systematic review

Acute type A aortic dissection repair in an octogenarian with achondroplasia: a case report

Application of thoracic endovascular aortic repair (TEVAR) in treating dwarfism with Stanford B aortic dissection

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