Cleidocranial dysplasia (CCD) is a rare genetic disorder characterized by skeletal abnormalities, including hypoplastic or absent clavicles, delayed closure of cranial sutures, and dental anomalies.

The procedural challenges encountered due to the patient's skeletal anomalies highlight the importance of individualized approach and careful consideration of anatomical variations in interventional cardiology procedures.

We present a case of a 72-year old female with a history of breast cancer treated with mastectomy and radio-chemotherapy with the port-a-catheter still in place in the left subclavian region. She presented to the emergency room with syncope. Upon examination, an electrocardiogram (EKG) revealed complete atrioventricular (AV) block with low-rate ventricular escape rhythm. The patient underwent temporary pacing via femoral access while awaiting definitive pacemaker implantation.

The absence of the right clavicle, first observed during pre-puncture fluoroscopy and later confirmed on post-procedure imaging (Fig. 1), significantly influenced the approach to pacemaker implantation. The right-sided approach for pacemaker insertion was the only option due to the presence of an existing port-a-cath on the left side, precluding a left-sided approach despite the anatomical anomaly of the absent right clavicle.

Future considerations for patients with cleidocranial dysplasia requiring cardiac device implantation should include comprehensive pre-procedural imaging, multidisciplinary collaboration involving cardiology and orthopedic specialists, and careful selection of vascular access routes based on individual anatomical variations